Publishing type：Research paper (scientific journal)   Publisher：International Heart Journal (Japanese Heart Journal)  

DOI： 10.1536/ihj.24-072

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Language：English   Publishing type：Research paper (scientific journal)  

The medical device market is a high-growth sector expected to sustain an annual growth rate of over 5%, even in developed countries. Daily, numerous patients have medical devices implanted or inserted within their bodies. While medical devices have significantly improved patient outcomes, as foreign objects, their wider use can lead to an increase in device-related infections, thereby imposing a burden on healthcare systems. Multiple materials with significant societal impact have evolved over time: the 19th century was the age of iron, the 20th century was dominated by silicon, and the 21st century is often referred to as the era of carbon. In particular, the development of nanocarbon materials and their potential applications in medicine are being explored, although the scope of these applications remains limited. Technological innovations in carbon materials are remarkable, and their application in medicine is expected to advance greatly. For example, diamond-like carbon (DLC) has garnered considerable attention for the development of antimicrobial surfaces. Both DLC itself and its derivatives have been reported to exhibit anti-microbial properties. This review discusses the current state of DLC-based antimicrobial surface development.

DOI： 10.3390/ijms25168593

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Language：English   Publishing type：Research paper (scientific journal)  

Controlling pulmonary blood flow in patients who have undergone Norwood palliation, especially early postoperatively, is challenging due to a change in the balance of systemic and pulmonary vascular resistance. We applied a combination therapy of clipping and balloon angioplasty for right ventricle-pulmonary artery (RV-PA) shunt to control pulmonary blood flow, but the influence of the combination therapy on the PA condition is uncertain. Retrospectively analysis was conducted of all infants with hypoplastic left heart syndrome who had undergone Norwood palliation with RV-PA shunt at Okayama University Hospital from January 2008 to September 2022. A total of 50 consecutive patients underwent Norwood palliation with RV-PA shunt in this study period. Of them, 29 patients underwent RV-PA shunt flow clipping, and the remaining 21 had unclipped RV-PA shunt. Twenty-three patients underwent balloon angioplasty for RV-PA shunt with clips. After balloon angioplasty, oxygen saturation significantly increased from 69 (59-76)% to 80 (72-86)% (p < 0.001), and the narrowest portion of the clipped conduit significantly improved from 2.8 (1.8-3.4) to 3.8 (2.9-4.6) mm (p < 0.001). In cardiac catheterizations prior to Bidirectional cavo-pulmonary shunt (BCPS), there were no significant differences in pulmonary-to-systemic flow ratio (Qp/Qs), ventricular end-diastolic pressure, Nakata index, arterial saturation, mean pulmonary artery pressure and pulmonary vascular resistance index. On the other hand, in Cardiac catheterizations prior to Fontan, Nakata index was larger in the clipped group (p = 0.02). There was no statistically significant difference in the 5-year survival between the two groups (clipped group 96%, unclipped group 74%, log-rank test: p = 0.13). At least, our combination therapy of clipping and balloon angioplasty for RV-PA shunt did not negatively impact PA growth. Although there is a trend toward better but not statistically significant difference in outcomes in the clipped group compared to the non-clipped group, this treatment strategy may play an important role in improving outcomes in hypoplastic left heart syndrome.

DOI： 10.1007/s00246-024-03579-6

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Language：English   Publishing type：Research paper (scientific journal)  

UNLABELLED: Sudden cardiac arrest during exercise can occur without prior warning signs at rest, highlighting the importance of monitoring for its prevention. To detect the signs of ischemic heart disease, including coronary artery anomalies, ST changes must be detected using three‑lead electrocardiograms (ECGs) corresponding to each region of the three coronary artery branches. We conducted ECG monitoring of five runners during a marathon using a wearable three‑lead ECG device (e-skin ECG; Xenoma Inc., Tokyo, Japan). Data without noise or artifacts were successfully collected for one of five runners during the entire marathon. Within the initial hour of the marathon, poor electrode adhesion to the skin hindered the data collection for the remaining four runners, which resulted in significantly decreased acquisition rate compared with the first hour (86.7 ± 13.4 % to 37.3 ± 36.9 %, p = 0.028). Couplets of premature ventricular contractions with clear ECG waveforms in the three leads were detected in one runner during the marathon. Further device improvements are necessary to enable marathon runners to obtain ECGs efficiently without affecting their performance. This study also demonstrated the potential applications of three‑lead wearable ECG monitoring for other short-duration sports and remote home-based cardiac rehabilitation. LEARNING OBJECTIVE: This is an initial trial of a three‑lead wearable electrocardiogram (ECG) monitoring device during a full marathon. ECG data were obtained with low noise and artifacts during the first hour of the marathon; however, the data acquisition rate decreased in the middle and late stages owing to poor electrode adhesion. This study demonstrated the possibility of applying wearable ECG monitoring during short-term exercise and cardiac rehabilitation to detect warning signs and prevent sudden cardiac arrest.

DOI： 10.1016/j.jccase.2024.03.004

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Language：English   Publishing type：Research paper (scientific journal)  

Staphylococcus aureus is one of the main causative bacteria for polyurethane catheter and artificial graft infection. Recently, we developed a unique technique for coating diamond-like carbon (DLC) inside the luminal resin structure of polyurethane tubes. This study aimed to elucidate the infection-preventing effects of diamond-like carbon (DLC) coating on a polyurethane surface against S. aureus. We applied DLC to polyurethane tubes and rolled polyurethane sheets with our newly developed DLC coating technique for resin tubes. The DLC-coated and uncoated polyurethane surfaces were tested in smoothness, hydrophilicity, zeta-potential, and anti-bacterial properties against S. aureus (biofilm formation and bacterial attachment) by contact with bacterial fluids under static and flow conditions. The DLC-coated polyurethane surface was significantly smoother, more hydrophilic, and had a more negative zeta-potential than did the uncoated polyurethane surface. Upon exposure to bacterial fluid under both static and flow conditions, DLC-coated polyurethane exhibited significantly less biofilm formation than uncoated polyurethane, based on absorbance measurements. In addition, the adherence of S. aureus was significantly lower for DLC-coated polyurethane than for uncoated polyurethane under both conditions, based on scanning electron microscopy. These results show that applying DLC coating to the luminal resin of polyurethane tubes may impart antimicrobial effects against S. aureus to implantable medical polyurethane devices, such as vascular grafts and central venous catheters.

DOI： 10.1007/s10047-023-01403-1

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Language：Japanese   Publisher：(一社)日本透析医学会  

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Language：English  

DOI： 10.1016/j.jtcvs.2023.07.040

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: A treatment strategy for congenital heart defects with moderate to severe pulmonary arterial hypertension (PAH) has not been established. OBJECTIVES: The purpose of this study was to identify patients in whom a treat and repair strategy was considered and to examine pretreatment variables associated with successful defect repair. METHODS: Patients with atrial or ventricular septal defect and PAH (pulmonary vascular resistance [PVR] ≥ 5 Wood units) eligible for the treat and repair strategy were included. Hemodynamics among pretreatment, pre-repair, and post-defect repair were compared. Clinical outcomes in patients with or without defect repair were also compared. Clinical outcomes included all-cause death, hospitalization for worsening pulmonary hypertension, and lung transplantation. RESULTS: Among 25 eligible for the treat and repair strategy, 20 underwent successful repair (repaired group) and 5 did not have a repair (unrepaired group). In the repaired group, PVR significantly decreased from 9.6 ± 2.6 WU at pretreatment to 5.0 ± 3.4 pre-repair (ß coefficient -4.6 [95% CI: -5.9 to -3.3]). The pulmonary to systemic blood flow ratio (Qp/Qs) increased from 1.5 ± 0.6 at pretreatment to 2.4 ± 1.3 pre-repair (ß coefficient 0.9 [95% CI: 0.4-1.38]). In the unrepaired group, pretreatment PVR decreased with treatment; however, PVR remained elevated. Qp/Qs did not change between pretreatment and post-treatment. The repaired group had a better prognosis than the unrepaired group (HR 0.092 [95% CI: 0.009-0.905]). Pretreatment mean pulmonary artery pressure, PVR, Qp/Qs, and arterial oxygen saturations were associated with undergoing defect repair. CONCLUSIONS: In this small cohort, a treat and repair strategy was successfully used in a significant proportion of the patients with congenital heart defects with moderate to severe PAH.

DOI： 10.1016/j.jacadv.2024.100887

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OBJECTIVES: There is limited information on long-term outcomes and trajectories of ventricular and valvular functions in patients with congenitally corrected transposition of the great arteries after anatomic repair according to the operative strategy with a median follow-up period of more than 10 years. METHODS: Twenty-nine patients who underwent anatomic repair in Okayama University Hospital between January 1994 and December 2020 were reviewed. Outcomes were compared between patients who underwent a double switch operation (DS group) and patients with an atrial switch with a Rastelli operation (Rastelli-Senning/Mustard group). RESULTS: Fifteen (52%) were in the DS group and 14 (48%) were in the Rastelli-Senning/Mustard group. The median follow-up period after anatomic repair was 12.7 (interquartile range 4.2-18.8) years. There were 3 (10%) early deaths and 3 (10%) late deaths. Survival rates for the entire cohort at 10 and 20 years were 86% and 71%, respectively, and were not different between the 2 groups. Using competing risk analysis, risks of heart failure, cardiac rhythm device implantation and atrial arrhythmia showed no significant differences between the 2 groups, whereas risk of reoperation was higher in the Rastelli-Senning/Mustard group than that in the DS group. Four patients after a DS operation and 1 patient after a Rastelli technique developed more than moderate aortic regurgitation. CONCLUSIONS: During a median follow-up period of more than 10 years, mortality rate and ventricular and valvular functions after anatomic repair were acceptable, though the incidences of late complications were relatively high, especially in the Rastelli-Senning/Mustard group.

DOI： 10.1093/icvts/ivae033

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.jtcvs.2023.07.003.

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(NPO)日本血管外科学会  

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Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

DOI： 10.1007/s12328-023-01892-w

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Other Link： https://link.springer.com/article/10.1007/s12328-023-01892-w/fulltext.html

Authorship：Last author  

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVES: The goal of this study was to identify the practical applications of intravenous cell therapy for single-ventricle physiology (SVP) by establishing experimental SVP models. METHODS: An SVP with a three-stage palliation was constructed in an acute swine model without cardiopulmonary bypass. A modified Blalock-Taussig (MBT) shunt was created using an aortopulmonary shunt with the superior and inferior venae cavae (SVC and IVC, respectively) connected to the left atrium (n = 10). A bidirectional cavopulmonary shunt (BCPS) was constructed using a graft between the IVC and the left atrium with an SVC cavopulmonary connection (n = 10). The SVC and the IVC were connected to the pulmonary artery to establish a total cavopulmonary connection (TCPC, n = 10). The survival times of half of the animal models were studied. The other half and the biventricular sham control (n = 5) were injected intravenously with cardiosphere-derived cells (CDCs), and the cardiac retention of CDCs was assessed after 2 h. RESULTS: All SVP models died within 20 h. Perioperative mortality was higher in the BCPS group because of lower oxygen saturation (P < 0.001). Cardiac retention of intravenously delivered CDCs, as detected by magnetic resonance imaging and histologic analysis, was significantly higher in the modified Blalock-Taussig and BCPS groups than in the TCPC group (P < 0.01). CONCLUSIONS: Without the total right heart exclusion, stage-specific SVP models can be functionally constructed in pigs with stable outcomes. Intravenous CDC injections may be applicable in patients with SVP before TCPC completion, given that the initial lung trafficking is efficiently bypassed and sufficient systemic blood flow is supplied from the single ventricle.

DOI： 10.1093/ejcts/ezad304

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Language：English  

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Publishing type：Research paper (scientific journal)   Publisher：Frontiers Media SA  

Aims

Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH.

Methods

This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged &amp;lt;20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death.

Results

The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD &amp;lt;2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45–13.73; P = .009).

Conclusions

The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.

DOI： 10.3389/fcvm.2023.1212882

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Authorship：Last author   Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Pulmonary artery coarctation (PACoA) is a major problem that increases the frequency of intervention. However, there is little evidence regarding the prediction of PACoA development. METHODS: A retrospective chart review was performed on 42 patients who underwent modified Blalock-Taussig shunt and preoperative contrast-enhanced computed tomography. An uneven PA branching was defined as an abnormal ductus arteriosus connection to the left PA distal to the PA branching on contrast-enhanced computed tomography. RESULTS: Nineteen (45.2%) of 42 patients were diagnosed with PACoA. The median diameters of the ductus on the aorta and PA sides were 4.1 mm and 3.6 mm in the PACoA group and 3.6 mm and 2.9 mm in the non-PACoA group, respectively (P = .07 and .28, respectively). Tortuous ductus was recognized in 7 (36.8%) patients in the PACoA group and 14 (60.8%) patients in the non-PACoA group (P = .12). PACoA was associated with pulmonary atresia (16 patients [84.2%] in the PACoA group and 12 patients [52.1%] in the non-PACoA group) (P = .02). All 19 patients had uneven PA branching in the PACoA group, whereas 5 of 23 (21.7%) patients had uneven PA branching in the non-PACoA group (P < .001). CONCLUSIONS: Uneven PA branching rather than the ductus arteriosus size was strongly associated with PACoA development; therefore, morphologic assessment by contrast-enhanced computed tomography should be considered in patients with pulmonary atresia.

DOI： 10.1016/j.jtcvs.2023.02.021

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Children with a congenital heart disease (CHD) are at a higher risk of developing epilepsy than the general population, but detailed characteristics of CHD-associated epilepsy have not been clarified. The purposes of this study were to determine the risk factors for developing epilepsy associated with CHD and to elucidate the characteristics of such epilepsy. METHODS: We performed a retrospective cohort study based on medical records of pediatric patients with CHD who were born between January 2006 and December 2016, underwent cardiac surgery at Okayama University Hospital, and were followed up until at least age three years. Multivariate logistic regression analysis was used to determine factors particularly associated with epilepsy occurrence. In patients who developed epilepsy, clinical data on seizure characteristics were further investigated. RESULTS: We collected data from 1024 patients, and 41 (4.0%) developed epilepsy. The presence of underlying disease (odds ratio [OR]: 2.413; 95% confidence interval [CI]: 1.150 to 4.883) and the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery score category 2 (OR: 4.373; 95% CI: 1.090 to 29.150) and category 5 (OR: 10.385; 95% CI: 1.717 to 89.016) were significantly related to epilepsy occurrence. Of the 41 patients with epilepsy, 15 (including nine with hypoplastic left heart syndrome) had focal impaired awareness seizures specified as autonomic seizures with vomiting, which tends to escape detection. CONCLUSIONS: We clarified the risk factors for developing epilepsy in children with CHD. We also found that autonomic seizure with vomiting is an important symptom in these children.

DOI： 10.1016/j.pediatrneurol.2023.07.004

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)  

Primary cardiac sarcomas are rare diseases with very poor prognoses. In this report, we present a case of coronary artery intimal sarcoma in a patient who survived for a long time after diagnosis. A 57-year-old female underwent percutaneous coronary intervention of the right coronary artery due to acute myocardial infarction caused by thrombotic occlusion and was diagnosed as having coronary artery intimal sarcoma. She underwent surgical resection and coronary artery bypass surgery of the artery, cryothermy coagulation, and postoperative adjuvant chemotherapy for 1 year. After 3 years, focal recurrence was detected in the caudal region of the left ventricular inferior wall. Radiotherapy was performed. The tumor shrank significantly after radiotherapy. Four years later, there was no significant abnormal uptake on positron-emission tomography/computed tomography. At 7 years after diagnosis, when this case report was submitted, the patient was alive and her performance had maintained a good status. Intimal sarcoma occurring in a coronary artery is extremely rare. The efficacy of treatments for cardiac intimal sarcoma, which include surgical resection, chemotherapy and radiotherapy, has been reported to be limited. To the best of our knowledge, this is the first report of a case of coronary artery intimal sarcoma with long-term survival after comprehensive therapies including surgical resection and radiotherapy.

DOI： 10.1536/ihj.22-578

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Language：English   Publishing type：Research paper (scientific journal)  

The aim of this study was to obtain comprehensive data regarding the hemocompatibility of diamond-like carbon (DLC)-coated expanded polytetrafluoroethylene (ePTFE). DLC increased the hydrophilicity and smoothened the surface and fibrillar structure, respectively, of the ePTFE. DLC-coated ePTFE had more albumin and fibrinogen adsorption and less platelet adhesion than uncoated ePTFE. There were scarce red cell attachments in in vitro human and in vivo animal (rat and swine) whole blood contact tests in both DLC-coated and uncoated ePTFE. DLC-coated ePTFE had a similar but marginally thicker band movement than uncoated-ePTFE with SDS-PAGE after human whole blood contact test. In addition, survival studies of aortic graft replacement in rats (1.5 mm graft) and arteriovenous shunt in goats (4 mm graft) were performed to compare the patency and clot formation between DLC-coated and uncoated ePTFE grafts. Comparable patency was observed in both animal models. However, clots were observed in the luminal surface of the patent 1.5 mm DLC-coated ePTFE grafts, but not in that of uncoated ePTFE grafts. In conclusions, hemocompatibility of DLC-coated ePTFE was high and comparable to that of uncoated ePTFE. However, it failed to improve the hemocompatibility of 1.5 mm ePTFE graft probably because increased fibrinogen adsorption canceled the other beneficial effects of DLC.

DOI： 10.1038/s41598-023-35594-7

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

歯科治療は,観血的処置にはみえなくても菌血症を起こすリスクが高い.今回,感染性心内膜炎(IE)高リスクに分類されるフォンタン手術後の患者が歯科治療に起因すると考えられる感染症を起こし,緊急入院に至る症例を経験した.患者は20歳の男性.多脾症候群,右室型単心室に対して,両側両方向性グレン手術とフォンタン手術の手術歴がある.2021年6月,近医で下顎左側第二大臼歯(#37)の慢性根尖性歯周炎の診断のもと,予防的抗菌薬の投与なく歯内治療を開始した.2021年7月,治療中の#37部の自発痛,悪寒,戦慄,発熱を自覚し,当院循環器内科を緊急受診した.履歴から歯性感染が疑われたため,当院歯周科へ緊急紹介され,#37急性根尖性歯周炎と診断した.IE高リスク患者のため緊急入院となり,経験的抗菌療法を開始した.入院5日目,抗菌薬持続投与下で#37の歯内治療を再開,入院12日目に歯内治療を終了,入院13日目に退院した.今回の症例を教訓に,患者自身が歯科治療に先立ち予防的抗菌薬投与の必要性を簡便に提示できる患者カードを作成した.本カードが適切に運用され,歯科治療由来のIE発症リスクが軽減されることを期待する.(著者抄録)

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

OBJECTIVES: The present study developed a new risk model for congenital heart surgery in Japan and determined the relationship between hospital procedural volume and mortality using the developed model. METHODS: We analyzed 47,164 operations performed between 2013 and 2018 registered in the Japan Cardiovascular Surgery Database-Congenital and created a new risk model to predict the 90-day/in-hospital mortality using the Japanese congenital heart surgery mortality categories and patient characteristics. The observed/expected ratios of mortality were compared among 4 groups based on annual hospital procedural volume (group A [5539 procedures performed in 90 hospitals]: ≤50, group B [9322 procedures in 24 hospitals]: 51-100, group C [13,331 procedures in 21 hospitals]: 101-150, group D [18,972 procedures in 15 hospitals]: ≥151). RESULTS: The overall mortality rate was 2.64%. The new risk model using the surgical mortality category, age-weight categories, urgency, and preoperative mechanical ventilation and inotropic use achieved a c-index of 0.81. The observed/expected ratios based on the new risk model were 1.37 (95% confidence interval, 1.18-1.58), 1.21 (1.08-1.33), 1.04 (0.94-1.14), and 0.78 (0.71-0.86) in groups A, B, C, and D, respectively. In the per-procedure analysis, the observed/expected ratios of the Rastelli, coarctation complex repair, and arterial switch procedures in group A were all more than 3.0. CONCLUSIONS: The risk-adjusted mortality rate for low-volume hospitals was high for not only high-risk but also medium-risk procedures. Although the overall mortality rate for congenital heart surgeries is low in Japan, the observed volume-mortality relationship suggests potential for improvement in surgical outcomes.

DOI： 10.1016/j.jtcvs.2022.06.009

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVES: We compared 2-patch repair (TP) with modified single-patch repair (MSP) for complete atrioventricular septal defects and evaluated their effect on the left atrioventricular valve (LAVV) competence. We also identified risk factors for unfavorable functional outcomes. METHODS: This retrospective study included 118 patients with complete atrioventricular septal defects who underwent intracardiac repair from 1998 to 2020 (MSP: 69; TP: 49). The median follow-up period was 10.4 years. The functional outcome of freedom from moderate or greater LAVV regurgitation (LAVVR) was estimated using the Kaplan-Meier method. RESULTS: The hospital mortality was 1.7% (2/118) and late mortality was 0.8% (1/118). Eight patients required LAVV-related reoperation (MSP: 4; TP: 4) and none required left ventricular outflow tract-related reoperation. In the MSP group without LAVV anomaly, the receiver operating characteristic curve analysis revealed that the ventricular septal defect (VSD) depth was strongly associated with moderate or greater postoperative LAVVR, with the best cutoff at 10.9 mm. When stratified according to the combination of intracardiac repair type and VSD depth, the MSP-deep VSD (VSD depth >11 mm) group showed the worst LAVV competence among the 4 groups (P = .002). According to multivariate analysis, weight <4.0 kg, LAVV anomaly, and moderate or greater preoperative LAVVR were independent risk factors for moderate or greater postoperative LAVVR, whereas MSP was not a risk factor. CONCLUSIONS: Postoperative LAVVR remains an obstacle to improved functional outcomes. MSP provides LAVV competence similar to TP unless deep VSD is present. The surgical approach should be selected on the basis of anatomical variations, specifically VSD depth.

DOI： 10.1016/j.jtcvs.2022.07.041

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DOI： 10.34376/jsachd.C-2022-0002

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Publishing type：Research paper (scientific journal)   Publisher：Cureus, Inc.  

DOI： 10.7759/cureus.32418

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Publishing type：Research paper (scientific journal)   Publisher：Texas Heart Institute Journal  

Anomalous origin of the left coronary artery from the opposite sinus of Valsalva with an intramural aortic course (L-ACAOS-IM) can cause syncope, sometimes as a prodrome of lethal events, including sudden cardiac death, in young athletes. The detailed mechanism of syncope in patients with L-ACAOS-IM is still unclear. This case report describes a 17-year-old boy who presented to the hospital because of syncope following chest pain with increasing frequency during exercise, such as playing soccer and running. In a treadmill exercise test, a decrease in blood pressure was seen (from 99/56 mm Hg to 68/38 mm Hg); chest pain and faintness accompanied by ST-segment elevation in lead aVR and ST-segment depression at other leads on electrocardiography were noted. These findings and symptoms disappeared spontaneously within a few minutes while clinicians prepared for emergency medications. Coronary computed tomography angiography (CCTA) showed that the origin of the left coronary artery (LCA) was the opposite sinus of Valsalva, and the course of the LCA was through the aortic wall toward the left coronary sinus. He was diagnosed with L-ACAOS-IM. After surgical treatment by unroofing the intramural part of the LCA and reconstructing a neo-ostium, he no longer experienced syncope during exercise. This case suggests that low cardiac output caused by myocardial ischemia, not life-threatening arrythmia, is a main mechanism of syncope in patients with L-ACAOS-IM. Consideration should be given to performing CCTA before an exercise stress test for young patients with syncope and chest pain to avoid the risk of severe myocardial ischemia.

DOI： 10.14503/thij-21-7677

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Language：English  

DOI： 10.1016/j.xjtc.2022.07.020

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Publishing type：Research paper (scientific journal)  

Although the freestyle aortic root bioprosthesis offers excellent clinical outcomes, the surgical mortality after reoperation is extremely high compared to that after aortic valve replacement and Bentall operation. We have described a surgical technique for structural valve deterioration of the freestyle bioprosthesis with a small aortic annulus. Modified Konno procedure with anterior aortic annular enlargement was indicated to repair the tentative pseudoaneurysm. Postoperative image of the aortic root showed a successful reconstruction without stenosis. This approach was found to be safe and efficient in this complicated reintervention case.

DOI： 10.23736/S0394-9508.21.05376-6

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Language：English   Publishing type：Research paper (scientific journal)  

We report 3 cases in sinus plication technique utilized to repair aortic or neoaortic root dilation and valve regurgitation after congenital heart disease operation.

DOI： 10.1093/ejcts/ezac456

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Language：Japanese   Publisher：(一社)日本外科学会  

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Publishing type：Research paper (scientific journal)   Publisher：Japanese Circulation Society  

DOI： 10.1253/circj.cj-22-0134

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Language：English  

Coronary artery aneurysm occurs in 0.3%-0.8% of patients with Kawasaki disease, and cases of rupture are extremely rare. Only 2 cases have been reported in which the patients survived. We report a case of ruptured coronary artery aneurysm that was treated with coronary artery bypass grafting and extracorporeal membrane oxygenation. (Level of Difficulty: Advanced.).

DOI： 10.1016/j.jaccas.2022.05.012

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.jtcvs.2021.08.083

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: We present our experience with transcatheter vascular occlusion using 0.035-inch hydrogel expandable coils, which has been reported only in a few cases in the pediatric cardiology fields. METHODS: This study is a retrospective analysis of all patients who underwent transcatheter embolization with 0.035-inch hydrogel coils at the Department of Pediatrics, Okayama University Hospital, between October 2018 and September 2020. RESULTS: Twenty patients with a median age of 5.1 years (0.05-26.0 years) and a median weight of 13.8 kg (3.0-56.8 kg) were included. A total of fifty-four 0.035-inch hydrogel coils, including 35 Azur 35 and nineteen Azur CX 35 coils (Terumo, Tokyo, Japan), were successfully deployed in 22 target vessels. The target vessels consisted of 10 aortopulmonary collaterals, 8 veno-venous collaterals, and 4 pulmonary arteriovenous malformations. We achieved technical success in all the target vessels. In total, the mean target vessel diameter was 4.4 mm, the mean number of 0.035-inch hydrogel coils was 2.5 per vessel. The mean device to vessel ratio was 1.6 for the anchor coil and 1.2 for the additional coil. Post-implantation angiograms revealed that the primary occlusion rate was 18/22 (82%). There were no periprocedural complications. CONCLUSIONS: The 0.035-inch hydrogel expandable coils are effective and safe in patients with congenital heart disease and vascular anomalies. These occlusion devices could be valuable options for interventional pediatric cardiologists.

DOI： 10.1016/j.jjcc.2022.04.005

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BACKGROUND: This study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot on pulmonary valve annulus growth, the rate of valve-sparing repair at the time of intracardiac repair, and long-term functional outcomes. METHODS: This retrospective study involved 330 patients with tetralogy of Fallot who underwent intracardiac repair between 1991 and 2019 and included 57 patients (17%) who underwent BTS. The mean follow-up period was 15.0 ± 7.3 years. We compared the data of patients who underwent BTS and patients who did not undergo BTS before intracardiac repair. RESULTS: The median age before BTS was 71 days (range, 28 to 199) and the median body weight was 4.3 kg (range, 3.3 to 6.8 kg). There were no inhospital or interstage deaths after BTS. The pulmonary valve annulus Z scores of patients with BTS revealed significant growth after BTS (from -4.2 ± 1.8 to -3.0 ± 1.7, P < .001). Valve-sparing repair was eventually performed in 207 patients (63%), including 26 (46%) who underwent staged repair. The overall freedom from pulmonary regurgitation-related reintervention was 99.7%, 99.1%, and 95.8% at 1, 5, and 20 years, respectively. CONCLUSIONS: A staged surgical strategy incorporating BTS as the first palliation for symptomatic patients resulted in no mortality. Blalock-Taussig shunt may have contributed to the avoidance of primary transannular patch repair and facilitated pulmonary valve annulus growth; therefore, approximately half of the symptomatic neonates and infants were recruited for valve-sparing repair. Staged repair may have led to functionally reliable delayed transannular patch repair, thereby resulting in fewer surgical reinterventions.

DOI： 10.1016/j.athoracsur.2021.01.087

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OBJECTIVE: Pulmonary artery coarctation may pose a risk for pulmonary stenosis and subsequent failure to achieve definitive repair. We sought to assess the impact of pulmonary artery coarctation on pulmonary artery growth. METHODS: A retrospective chart review was performed in 130 patients, including 37 single ventricles with a modified Blalock-Taussig shunt as first palliation. Pulmonary artery coarctation was defined as discrete stenosis of the pulmonary artery, with a diameter of less than 3 mm and with the ductus arteriosus connected. Preoperative echocardiography showed pulmonary artery coarctation in 29 patients (22%). Concomitant pulmonary artery plasty was performed in 14 patients with discrete stenosis having a diameter of less than 2 mm. RESULTS: Pre-modified Blalock-Taussig shunt left pulmonary artery z-scores were lower in patients with pulmonary artery coarctation than in those without (-4.0 [-5.8, -2.1] vs -1.7 [-2.6, -0.8], P < .001), and this remained the same even after modified Blalock-Taussig shunt (-2.5 [-5.1, -0.5] vs -0.5 [-2.4, 0.8], P = .010). Concomitant pulmonary artery plasty did not result in catch-up growth of the left pulmonary artery (post-modified Blalock-Taussig shunt left pulmonary artery z-score in patients with pulmonary artery plasty: -3.0 (-6.5, -2.0) versus those without: -1.8 (-3.3, -0.3), P = .279). Definitive repair/Fontan completion was achieved in 111 patients (85%), and this was not affected by the presence of pulmonary artery coarctation. CONCLUSIONS: Pulmonary artery coarctation affected disproportionate pulmonary artery growth throughout the staged repair, but did not result in failure of definitive repair/Fontan completion. Pulmonary artery plasty during the neonatal period did not contribute to catch-up growth of the left pulmonary artery; therefore, surgical indications and timing should be carefully considered.

DOI： 10.1016/j.jtcvs.2021.09.070

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1177/02184923221094958

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Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

DOI： 10.1007/s00246-021-02782-z

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Other Link： https://link.springer.com/article/10.1007/s00246-021-02782-z/fulltext.html

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Sudden cardiac accident (SCA) during a marathon is a concern due to the popularity of the sport. Preventive strategies, such as cardiac screening and deployment of automated external defibrillators have controversial cost-effectiveness. We investigated the feasibility of use of a new electrocardiography (ECG) sensor-embedded fabric wear (SFW) during a marathon as a novel preventive strategy against SCA. Twenty healthy volunteers participated in a full marathon race. They were equipped with a SFW hitoe® with a transmitter connected via Bluetooth to a standard smartphone for continuous ECG recording. All data were stored in a smartphone and used to analyze the data acquisition rate. The adequate data acquisition rate was > 90% in 13, 30-90% in 3, and < 10% in 4 runners. All of 4 runners with poorly recorded data were female. Inadequate data acquisition was significantly associated with the early phase of the race compared with the mid phase (P = 0.007). Except for 3 runners with poor heart rate data, automated software calculation was significantly associated with manual analysis for both the mean (P < 0.001) and maximum (P = 0.014) heart rate. We tested the feasibility of continuously recording cardiac data during a marathon using a new ECG sensor-embedded wearable device. Although data from 65% of runners were adequately recorded, female runners and the early phase of the race tended to have poor data acquisition. Further improvements in device ergonomics and software are necessary to improve ability to detect abnormal ECGs that may precede SCA.

DOI： 10.1007/s00380-021-01939-3

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OBJECTIVE: Repair of total anomalous pulmonary venous connection (TAPVC) in neonates with right atrial isomerism and functional single ventricle is challenging. In our novel strategy, primary draining vein stenting (DVS) was applied to patients with preoperative pulmonary vein obstruction to delay TAPVC repair. This study investigated our initial experience with a strategy of delayed TAPVC repair, incorporating DVS. METHODS: Twenty-nine patients with right atrial isomerism and functional single ventricle who had a severe obstruction in the course of draining veins, who required surgical or catheter intervention in their neonatal period were retrospectively reviewed (primary DVS: n = 11; primary TAPVC repair: n = 18). RESULTS: Patients in the primary DVS group had more mixed type TAPVC (primary DVS: n = 5, 45.5%; primary TAPVC repair: n = 2, 11.1%; P = .03) and required more systemic to pulmonary shunt surgeries during their lifetime (primary DVS: n = 9, 81.8%; primary TAPVC repair: n = 6, 33.3%; P = .047). Kaplan-Meier analysis showed that primary DVS repair was associated with improved survival compared with primary TAPVC repair (survival rates at 90 days, 1 year, 3 years and 5 years: primary DVS: 100%, 80%, 68.6%, and 54.9%; primary TAPVC repair: 55.6%, 38.9%, 38.9%, and 38.9%, respectively [P = .04]). Of the 4 patients who underwent stenting of the ductus venosus, 3 had elevated liver enzymes after surgical repair of TAPVC due to ductus venosus steal, which markedly improved after coil embolization of the stent. CONCLUSIONS: For neonates with obstructive TAPVC and functional single ventricle, our delayed TAPVC repair using primary DVS appeared to improve survival compared with the conventional strategy.

DOI： 10.1016/j.xjon.2021.11.012

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Surgical restoration of subvalvular geometry is very important to prevent recurrence of ischemic/functional mitral regurgitation. We evaluated an approach to the radical repair of left ventricular (LV) remodeling for three different cases with responsible coronary lesions. Leaflet tethering was corrected by tugging of the papillary-ventricular complex, which consists of the base of papillary muscles and posterior LV wall. The main lesion of the postinfarction scar was concomitantly excluded. Restoration of LV remodeling diminished mitral regurgitation with minimal leaflet tethering and improved systolic LV function. This technique may be an aggressive and encouraged approach for patients with ischemic/functional mitral regurgitation.

DOI： 10.1016/j.athoracsur.2021.03.095

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Language：Japanese   Publisher：日本成人先天性心疾患学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：JAPANESE SOC MEDICAL & BIOLOGICAL ENGINEERING  

Cardiac arrest has been reported during participation in several sports. Of these sports, marathon running is a particularly popular sport but imposes high cardiac load. Indeed, its popularity has been growing worldwide. Risk of cardiac arrest during marathon races is also expected to increase. Several studies have recorded electrocardiographic (ECG) information during marathon races to protect athletes from cardiac arrest. Although evaluable ECG data have been obtained and analyzed, cost-effectiveness of the system, data quality, and clinical significance remain inadequate. This report is the first to describe an economical electrocardiograph built into a T-shirt for use during marathon race. Twenty healthy runners aged 20 to 59 years (mean 36 years) wore the ECG device while running. The ECG data were monitored and analyzed to assess the observed frequencies of specified arrhythmias and the sections of the marathon in which the arrhythmias occurred. Of the ECG data obtained from 14 runners who completed the full marathon, six ECG datasets were evaluable. In some runners, there was inadequate contact between the electrode and body surface or poor Bluetooth connection between the ECG wireless transmitter and smartphone. Regarding arrhythmia analysis, all evaluable data that were analyzed showed some rhythm fluctuations. In conclusion, this economical T-shirt type ECG sensor provided evaluable ECG data during marathon races, although the evaluable rate was not high. The data were used to analyze specified arrhythmias, but some difficulties were encountered. The ECG sensor did not function properly because of a system error. The ECG sensor was not adequately moistened to record ECGs accurately. Moreover, some runners chose an unsuitable shirt size, which impaired the stability and strength of the electrode-skin contact. These shortcomings produced noise in the ECG data, which made it difficult to analyze arrhythmias. The next step will be to solve these problems and acquire data from a large number of runners.

DOI： 10.14326/abe.11.151

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Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1053/j.optechstcvs.2022.06.008

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Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1053/j.optechstcvs.2022.03.003

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Language：English   Publishing type：Research paper (scientific journal)  

The number of patients with adult congenital heart disease (ACHD) has been dramatically increasing and adults with congenital heart disease now outnumber children with congenital heart disease. However, patients with ACHD are still at increased risk of morbidity and mortality due to residua and sequelae. Although echocardiography is an indispensable imaging modality in the comprehensive assessment of ACHD, accurate echocardiographic assessment of ACHD is challenging especially for physicians or sonographers who are not familiar with ACHD because of its complex morphology, physiology, and hemodynamics. A recently developed fusion imaging technology can provide synchronized display of real-time echocardiographic images and multiplanar reconstruction images of computed tomography or magnetic resonance imaging corresponding to the image plane of real-time echocardiography. We have reported the clinical utility of this fusion imaging technology for the precise evaluation of complex ACHD. On the other hand, conventional echocardiographic technology also plays an important role in assessing unique ACHD pathophysiology. For example, restrictive right ventricular physiology is a common finding after tetralogy of Fallot or pulmonary stenosis repair and can be evaluated by conventional pulsed-wave Doppler. In this review, we discuss the clinical usefulness of modern and conventional echocardiographic technologies for the evaluation of ACHD by presenting a case series.

DOI： 10.1007/s12574-021-00533-w

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Language：Japanese   Publisher：(一社)日本外科学会  

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OBJECTIVES: This study aimed to evaluate whether recoarctation of the aorta (reCoA) after the Norwood procedure for hypoplastic left heart syndrome correlates with pre- and postoperative anatomic factors. METHODS: This retrospective study included 48 patients who underwent Norwood procedure with right ventricle-to-pulmonary artery conduit between 2009 and 2017. Anatomical factors such as preoperative length, diameter of the main pulmonary artery (MPA), and postoperative neoaortic arch angle stratified by arch reconstruction technique were analysed using the receiver operating characteristic analysis. RESULTS: Eleven patients needed surgical intervention for reCoA at stage 2. Out of the 30 patients who underwent direct anastomosis during arch reconstruction, 7 developed reCoA. Seven patients received the full patch augmentation (patch augmentation for both lesser and greater curvatures) and were all spared from reCoA. Among the patients who had direct anastomosis, the preoperative MPA length was correlated with the postoperative arch angle (P = 0.021) and was associated with the occurrence of reCoA (P = 0.002) and the best cutoff value for MPA length was 10 mm. The postoperative arch angle was also correlated with the incidence of reCoA (P < 0.001) and was larger in patients who underwent the full patch augmentation than in patients who had direct anastomosis (126° vs 112°, P = 0.005) despite comparable MPA length. CONCLUSIONS: ReCoA after the Norwood procedure correlates with MPA length when a direct anastomosis was used. Direct anastomosis can be considered in patients with a longer preoperative MPA. In other cases, the full patch augmentation should be considered for obtaining a large and smooth neoaortic arch.

DOI： 10.1093/icvts/ivab170

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Language：Japanese   Publisher：(一社)日本胸部外科学会  

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We report a case of ventricular septal defect (VSD) in which we attempted to treat pulmonary arterial hypertension (PAH) with the goal of VSD closure in an adult with suspected Eisenmenger syndrome in childhood. Four years previously (age 41 years), she was referred to our department due to repeated hemoptysis requiring further treatment of PAH. We started combination therapy with several pulmonary vasodilators. Two years later, her pulmonary vascular resistance (PVR) was improved but still not at the level where VSD closure was possible. To control the increased PA flow resulting from intensive PAH treatment and to reduce the risk of hemoptysis, we performed pulmonary artery banding (PAB). As the risk of hemoptysis decreased, a prostacyclin analog was introduced, and the dose was increased. More than 1 year after PAB, active vasoactivity testing became positive, suggesting that the pulmonary vascular lesion was now "reversible". We performed VSD closure and atrial septal defect creation even though her PVR was still high. After the operation, her exercise capacity was remarkably improved. We suggest that stepwise surgical repair with pulmonary vasodilators is an important treatment option for select patients with VSD with severe PAH. <Learning objective: Advances in pulmonary arterial hypertension (PAH) treatment have led to the use of a "treat-and-repair" strategy to close the intracardiac shunt after PAH treatment in select patients with adult congenital heart disease. In our case, ventricular septal defect (VSD) closure was achieved with stepwise surgical repair and a combination of pulmonary vasodilators, even though long-standing severe PAH with persistent hemoptysis remained. Even after a long period of exposure to high blood flow, this strategy may reduce pulmonary vascular resistance and permit eventual closure of the VSD.>.

DOI： 10.1016/j.jccase.2021.02.013

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND AND AIMS: Our study aimed to establish safer methods to manage home blood transfusion by using a remote vital signs data monitoring system. Home care is administered for patients with various medical disorders; however, home blood transfusion remains challenging owing to the risk of transfusion-related complications. METHODS: We set up a remote vital signs data monitoring system to improve the safety of home blood transfusions. Using an Internet-based vital signs data monitoring system, the heart rate, electrocardiography, respiration rate, and percutaneous oxygen saturation (SpO2) were monitored and recorded during the entire home transfusion period. RESULTS: Ten transfusions in three patients were monitored; two of the patients had an abnormality in a single vital sign (decreased SpO2 decrease and increased respiratory rate); these were not transfusion-related complications. Vital sign anomalies also occur because of errors in using the measurement device and noise associated with body movements. The presence of abnormalities in at least two vital signs among SpO2 decrease, tachycardia, and increased respiratory rate that persisted for >5 minutes was defined as a complicated vital sign abnormality (CVSA). There were no severe transfusion-related complications with CVSA in the present study. CONCLUSION: This study indicates the feasibility and sustainability of real-time remote monitoring of vital signs for the safety of home transfusion. Although CVSA may function as an indicator of severe transfusion-related complications, these findings need to be confirmed with further studies.

DOI： 10.1002/hsr2.380

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Language：English  

Aortic root abscess is one of the most catastrophic complications of aortic valve endocarditis. Complete debridement is recommended regardless of the kind of infected lesion. A 37-year-old man with extensive aortic root abscess due to prosthetic aortic valve endocarditis was surgically treated. The main lesion was the aorto-mitral continuity extending to the commissure between the left and right coronary cusps. After debridement, the aortic annulus underneath the left coronary artery was reconstructed using a handmade aorto-mitral monobloc valve without aortic annuloplasty. This valve is required for the extensive root abscess of the left and non-coronary sinus to achieve complete debridement.

DOI： 10.1016/j.athoracsur.2020.11.046

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OBJECTIVES: To reveal the mid-term outcomes of Contegra implantation for the reconstruction of the right ventricular outflow tract to proximal branch pulmonary arteries in a multicentre study. METHODS: Between April 2013 and December 2019, 178 Contegra conduits were implanted at 5 Japanese institutes. The median age and body weight at operation were 16 months (25th-75th percentile: 8-32) and 8.3 kg (6.4-10.6). Sixteen patients were neonates (9.0%). Selected conduit sizes were 12 mm in 28 patients (15.7%), 14 mm in 67 patients (37.6%), 16 mm in 66 patients (37.1%), 18 mm in 5 patients (2.8%) and <12 mm in 12 patients (6.7%). Fifty-six grafts (31.4%) were ring supported. Proximal branch pulmonary arteries were concomitantly augmented in 85 patients (47.5%). Follow-up was completed in all patients and the median follow-up period was 3.1 years (1.3-5.1). RESULTS: The overall, conduit explantation-free and conduit infection-free survival rates at 5 years were 91.3%, 71.0% and 83.7%, respectively. Infection (P = 0.009) and common arterial trunk (P = 0.024) were risk factors for explantation. Conduit durability was shorter in smaller one (P < 0.001). Catheter interventions (for conduit to proximal branch pulmonary artery)-free survival rates at 5 years was 52.9%; however, need for catheter interventions was not a risk factor for conduit explantation. CONCLUSIONS: Mid-term outcomes of reconstruction of the right ventricular outflow tract to the proximal branch pulmonary arteries with Contegra were acceptable. The need for explantation over time was higher in smaller conduits. Conduit infection was a strong risk factor for conduit explantation. Frequently and repeated catheter interventions effectively extended the conduit durability.

DOI： 10.1093/icvts/ivab075

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BACKGROUND: This study aimed to determine the factors related to reintervention, especially for pulmonary stenosis (PS), in patients with Taussig-Bing anomaly (TBA) after the arterial switch operation. METHODS: This retrospective study included 34 patients with TBA who underwent the arterial switch operation between 1993 and 2018. Preoperative anatomic and physiologic differences and long-term outcomes were determined using a case-matched control with transposition of the great arteries with ventricular septal defect and TBA with an anterior and rightward aorta. RESULTS: At arterial switch operation, median age was 43 days (range, 16 to 102) and median body weight was 3.6 kg (range, 2.8 to 3.8 kg). Aortic arch obstruction and coronary anomalies were present in 64% and 41% of patients, respectively. The hospital mortality rate was 11%, including one cardiac death, and the late mortality rate was 2.9%. Furthermore, 41% patients underwent 26 reinterventions for PS. Patients undergoing PS-related reintervention had a significantly larger native pulmonary artery to aortic annulus size ratio than patients not receiving reintervention (1.69 vs 1.41, P = .02). This ratio was the only predictor of PS-related reintervention; it was significantly higher in the TBA group than in the transposition of great arteries/ventricular septal defect group. The PS-related reintervention was required more in the TBA group than in the transposition of great arteries/ventricular septal defect group. CONCLUSIONS: Regardless of complex coronary anatomy and associated anomalies, early and late survival were acceptable. Postoperative PS was strongly associated with having a larger native pulmonary valve, suggesting that an optimal surgical reconstruction was required for achieving an appropriate aortopulmonary anatomic relationship during the arterial switch operation.

DOI： 10.1016/j.athoracsur.2020.06.016

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OBJECTIVES: The aim of this study was to investigate whether milrinone infusion improved one-year survival in patients who underwent the Norwood-Sano procedure. DESIGN: A retrospective observational study. SETTING: A single-institution university hospital. PARTICIPANTS: Children who underwent the Norwood-Sano procedure from January 2008 to December 2014. INTERVENTIONS: Patients were categorized into two groups: group E+D, who received routine epinephrine and dopamine infusion, and group M, who received routine milrinone infusion for intra- and postoperative inotropic support. MEASUREMENTS AND MAIN RESULTS: The primary outcome of this study was one-year survival after the Norwood-Sano procedure. A total of 45 patients were included (group E+D, 22; group M, 23). One-year survival in group M was significantly higher than that in group E+D (95.7% [22/23] v 72.7% [16/22], p = 0.03). A Kaplan-Meier curve also showed that one-year survival in group M was significantly higher than that in group E+D (p = 0.04), from the result of the log-rank test. The number of patients who had any arrhythmias in the intensive care unit (ICU) was significantly lower in group M than in group E+D (21.7% [5/23] v 50% [11/22], p = 0.03). The duration of ICU stay did not have statistical difference between groups (group M 19; interquartile range [IQR], 15-28) v group E+D 19.5 (IQR, 16.3-35.5) days, p = 0.57). CONCLUSIONS: Perioperative milrinone infusion improved the mortality after the Norwood-Sano procedure. Potential advantages of milrinone compared with epinephrine are fewer arrhythmias and better systemic perfusion, which could decrease lethal cardiac events in the ICU.

DOI： 10.1053/j.jvca.2021.02.017

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Authorship：Lead author   Language：Japanese   Publisher：(有)科学評論社  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：ROYAL SOC CHEMISTRY  

Pathological mineralization, such as that in atherosclerosis or following transplantation of biomaterials, is associated with millions of deaths worldwide. A deeper understanding of the mechanisms underlying mineral nucleation in pathological mineralization will facilitate the development of novel bioinspired materials/techniques as therapeutics for either disease prevention or bone tissue reconstruction. The objective of this study was to perform a systematic and ultrastructural analysis of the nucleation sites of atherosclerotic mineralization from the viewpoint of material science. Histological staining, ultrastructural analysis, and selected area elemental mapping of human atherosclerotic tissues revealed two different nucleation sites in a collagenous and a lipidic area, respectively, comprising phospholipidic large vesicles (average 827 +/- 470 nm) and non-vesicular phospholipids. In both sites, the initial minerals were identified to be amorphous calcium phosphate (ACP), which then transformed into hydroxyapatite (HAp). Mineralization assays in vitro was performed by incubating non-vesicular low- (LDL) and high- (HDL) density lipoprotein and cholesterol in 2 mM CaCl2 for 2 days, without additional phosphate. Calcium ions in the formed minerals, measured using atomic absorption (AA) spectroscopy, showed that only LDL was mineralized. We then evaluated the mineralization of the major phospholipids, phosphatidylserine (PS) and phosphatidylcholine (PC) under the same conditions. The results of AA and mineral dry weight showed that PS was more abundantly mineralized than PC, most likely due to its higher hydrolysis rate. Similar to the in vivo findings, PS mineralization initially formed ACP, which then crystallized into HAp. Taken together, these results indicated that atherosclerosis involves two distinct nucleation sites and that non-vesicular phospholipids were one of the major nucleation sites for atherosclerotic mineral formation.

DOI： 10.1039/d1ma00273b

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BACKGROUND: Transvenous lead extraction (TLE) is necessary because of system infection, lead malfunction, or system upgrade. Patients with severe left ventricular dysfunction (SLVD) undergoing TLE may be at a higher risk because hemodynamic parameters may change unfavorably during or after TLE; however, this has not yet been clarified. OBJECTIVE: The purpose of this study was to examine whether patients with SLVD undergoing TLE have higher mortality. METHODS: All patients who underwent TLE were stratified as follows: patients with ejection fraction ≤ 35% (SLVD group) and those with ejection fraction > 35% (non-SLVD group). RESULTS: We assessed the data of 200 patients (SLVD group, 36; non-SLVD group, 164). Brain natriuretic peptide level and cardiac resynchronization therapy rate were higher in the SLVD group than in the non-SLVD group. There were no significant between-group differences in major complications and clinical success rates. Patients with SLVD were more likely to require additional hemodynamic support, such as catecholamine infusion, temporary atrial-ventricle sequential pacing, and temporary cardiac resynchronization therapy pacing (27.8% vs 1.2%; P < .001). The survival rate was not significantly different between the groups at 30 days and 1 year after TLE (SLVD vs non-SLVD: 30 days: 97.2% vs 99.4%; P = .215; 1 year: 80.6% vs 91.5%; P = .053). Multivariate Cox regression analysis revealed log brain natriuretic peptide and serum hemoglobin levels as predictors for 1-year mortality. CONCLUSION: The prognosis after TLE was comparable between patients with and without SLVD. However, additional hemodynamic support was often necessary for patients with SLVD.

DOI： 10.1016/j.hrthm.2021.01.026

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Primary cardiac tumor can arise from any location in the right and left cardiac chamber. Complete excision is generally recommended because of uncertainty regarding malignancy; however it is important to minimize the resultant functional deterioration after surgery. We report a case of endocardial hemangioma (4 × 3 × 3 cm) on the free wall of the right ventricle, located between the anterior and posterior papillary muscles. We describe details of the procedure to preserve the right ventricular volume and competence of the tricuspid valve.

DOI： 10.1016/j.athoracsur.2020.09.078

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OBJECTIVES: The goal of this study was to investigate the long-term outcome of aortic valve replacement (AVR) for severe aortic insufficiency with a focus on pre- and postoperative left ventricular (LV) function to explore predictive factors that influence the recovery of LV function and clinical outcome. METHODS: A total of 478 patients who underwent AVR for pure severe aortic insufficiency were grouped according to the preoperative echocardiographical LV ejection fraction (EF): low (LO) EF <35% (n = 43), moderate EF 35-50% (n = 150) or normal EF >50% (n = 285). RESULTS: Actuarial survival at 10 years post-AVR was 64% with a LO EF, 92% with a moderate EF and 93% with a normal EF (P = 0.016), whereas 10-year rates of freedom from major adverse cerebral and cardiovascular events were 47%, 79% and 84%, respectively (P < 0.0001). Echocardiography at 1 year post-AVR demonstrated that EF substantially improved in all groups. We noted a significant difference in survival (P = 0.0086) and in freedom from major adverse cerebral and cardiovascular events (P = 0.024) between patients with an EF ≥35% and those with an EF <35% in the LO EF group. The multivariable logistic regression model showed that predictive factors for lack of improvement in EF 1 year post-AVR in the LO EF group included plasma brain natriuretic peptide >365 pg/mL (P = 0.0022) and echocardiographic LV mass index) >193 g/m2 (P = 0.0018). CONCLUSIONS: Long-term outcome post-AVR for severe aortic insufficiency was largely influenced by preoperative LV function. Predictive factors of failure to recover ventricular function post-AVR included EF <25%, pre-brain natriuretic peptide >365 pg/mL or LV mass index >193 g/m2.

DOI： 10.1093/icvts/ivab020

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Takeuchi repair is a unique surgical approach in anomalous left coronary artery from the pulmonary artery. We present an adult patient with anomalous left coronary artery from the pulmonary artery with multiple late structural complications after Takeuchi repair who was evaluated using multimodality imaging, including newly developed cardiac fusion imaging with computed tomography and echocardiography. (Level of Difficulty: Advanced.).

DOI： 10.1016/j.jaccas.2021.02.035

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DOI： 10.1093/ejcts/ezaa483

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Few reports on a biventricular working heart model with ex vivo perfusion exist owing to the complexity of establishing a circuit. Hence, we investigated it for donation after circulatory death. The heart in six juvenile pigs (~20 kg) was arrested by asphyxiation. After 30 minutes of global ischemia, the heart was harvested, reperfused with normoxemic blood cardioplegia for 20 minutes, and subsequently perfused with hyperxemic blood. After 70 minutes of controlled reperfusion, the system was switched to the biventricular working mode. Cardiac function was assessed before anoxia and during the biventricular mode. Left and right ventricular functions worsened during the biventricular mode, as compared to those before anoxia (dP/dtmax , 673 ± 120 vs. 283 ± 95 and 251 ± 35 vs. 141 ± 21 mm Hg/s, respectively; P < .001). Systemic (resistance/100 g net heart weight) and pulmonary vascular resistance indexes during the biventricular mode were similar to those before anoxia (829 ± 262 vs. 759 ± 359, P = .707, and 167 ± 57 vs. 158 ± 83 dynes·sec·cm-5 - l-100-g net heart weight, P = .859, respectively). The biventricular working heart model with ex vivo perfusion was feasible, exhibited stable hemodynamics, and has the potential to be a powerful tool for direct cardiac function assessment.

DOI： 10.1111/aor.13834

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In donation after circulatory death heart transplantation, the donor heart is exposed to circulatory load. The right ventricle, due to its structure, has high compliance for volume load but is particularly vulnerable to increased pressure load. This study used a porcine model to conduct a functional assessment of the hemodynamics of the heart, with a focus on the right ventricle. Six pigs weighing 24.6 ± 1.4 kg were used. Circulatory death was induced by asphyxiation after median sternotomy. After 30 minutes in the state of global warm ischemia, the ascending aorta was clamped, followed by a 20-minute reperfusion of the heart with a 20°C blood cardioplegia solution. Systemic circulation was established by cardiopulmonary bypass after aortic cross-clamping. After initial reperfusion, the blood cardioplegia solution was replaced with blood. The blood was then rewarmed while the heart was still in a non-working state. Cardiac function was assessed twice in situ, first by the thermodilution method, and then, by the pressure-volume measurement both at preischemia and at three hours after initiation of reperfusion. The recovery rate of cardiac output was 75%. End-systolic elastance (P = .02) and pulmonary arterial elastance significantly increased (P = .03), but the ratio of arterial elastance to end-systolic elastance was preserved (P = .91) in the right ventricle. Despite a decrease in cardiac output after reperfusion from warm ischemia, the right ventricle had a potential to respond the elevated afterload. It is important that donations after circulatory death heart transplantation should be performed with attention to avoiding right ventricular distension.

DOI： 10.1111/aor.13823

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AIMS: Angiotensin II (AngII) is a potential contributor to the development of abdominal aortic aneurysm (AAA). In aortic vascular smooth muscle cells (VSMCs), exposure to AngII induces mitochondrial fission via dynamin-related protein 1 (Drp1). However, pathophysiological relevance of mitochondrial morphology in AngII-associated AAA remains unexplored. Here, we tested the hypothesis that mitochondrial fission is involved in the development of AAA. METHODS AND RESULTS: Immunohistochemistry was performed on human AAA samples and revealed enhanced expression of Drp1. In C57BL6 mice treated with AngII plus β-aminopropionitrile, AAA tissue also showed an increase in Drp1 expression. A mitochondrial fission inhibitor, mdivi1, attenuated AAA size, associated aortic pathology, Drp1 protein induction, and mitochondrial fission but not hypertension in these mice. Moreover, western-blot analysis showed that induction of matrix metalloproteinase-2, which precedes the development of AAA, was blocked by mdivi1. Mdivi1 also reduced the development of AAA in apolipoprotein E-deficient mice infused with AngII. As with mdivi1, Drp1+/- mice treated with AngII plus β-aminopropionitrile showed a decrease in AAA compared to control Drp1+/+ mice. In abdominal aortic VSMCs, AngII induced phosphorylation of Drp1 and mitochondrial fission, the latter of which was attenuated with Drp1 silencing as well as mdivi1. AngII also induced vascular cell adhesion molecule-1 expression and enhanced leucocyte adhesion and mitochondrial oxygen consumption in smooth muscle cells, which were attenuated with mdivi1. CONCLUSION: These data indicate that Drp1 and mitochondrial fission play salient roles in AAA development, which likely involves mitochondrial dysfunction and inflammatory activation of VSMCs.

DOI： 10.1093/cvr/cvaa133

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BACKGROUND: Congenital heart disease (CHD) is the most common neonatal and pediatric cardiac indication for extracorporeal membrane oxygenation (ECMO). Risk factors of survival and neurologic complication were different in many centers. We sought to evaluate survival and neurological outcome after ECMO in patients with CHD. METHODS: We retrospectively reviewed the medical records of 37 patients (<16 years old) who received ECMO. Indications for ECMO were failure to wean from cardiopulmonary bypass in 18 patients, extracorporeal cardiopulmonary resuscitation (ECPR) in 13 patients, and others in 6 patients. The median cardiopulmonary resuscitation (CPR) duration in ECPR patients was 48 min (interquartile range: 38-53 min). Neurological outcomes were evaluated using the Pediatric Cerebral Performance Category (PCPC) scale one year after hospital discharge. RESULTS: The median ECMO duration was 160 (91-286) h. Twenty-nine patients (78%) were successfully weaned off ECMO. Overall survival to hospital discharge was 59%. Risk factors of mortality were as follows: ECMO duration >1 week and urine output <1 mL/kg/h in the first 24 h after ECMO induction by multivariable analysis. Of the 22 survivors, 15 (68%) patients had a favorable outcome (PCPC ≤2). Risk factors for unfavorable outcomes (PCPC ≥3) included ECPR as indication and CPR of longer than 40 min. CONCLUSIONS: Longer ECMO duration and lower urine output were associated with increased mortality. Neurologic outcomes were not satisfactory when CPR was required for a longer period before ECMO establishment.

DOI： 10.1177/0218492321997379

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Bovine jugular vein (BJV) and expanded polytetrafluoroethylene (ePTFE) conduits have been described as alternatives to the homograft for right ventricular outflow tract (RVOT) reconstruction. This study compared RVOT reconstructions using BJV and ePTFE conduits performed in a single institution. The valve functions and outcomes of patients aged < 18 years who underwent primary RVOT reconstruction with a BJV or ePTFE conduit between 2013 and 2017 were retrospectively investigated. 44 patients (20 and 24 with BJV and ePTFE conduits, respectively) met the inclusion criteria. The mean follow-up time was 4.5 ± 1.5 years. No significant differences in peak RVOT velocity (1.8 ± 0.9 m/s vs 2.1 ± 0.9 m/s, P = 0.27), branch pulmonary stenosis (P = 0.50), or pulmonary regurgitation (P = 0.44) were found between the BJV and ePTFE conduit groups, respectively. Aneurysmal dilatation of the conduit was observed in 25.0% of the patients in the BJV conduit group but not in the ePTFE conduit group (P = 0.011). All the cases with aneurysmal dilatation of the BJV conduit were complicated with branch pulmonary stenosis up to 3.0 m/s (P = 0.004). No conduit infections occurred during the follow-up period, and no significant difference in conduit replacement (20.0% vs 8.3%, P = 0.43) was found between the BJV and ePTFE conduit groups, respectively. The outcomes of the RVOT reconstructions with BJV and ePTFE conduits were clinically satisfactory. Aneurysmal dilatation was found in the BJV conduit cases, with branch pulmonary stenosis as the risk factor.

DOI： 10.1007/s00246-020-02458-0

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Although cardiosphere-derived cells (CDCs) improve cardiac function and outcomes in patients with single ventricle physiology, little is known about their safety and therapeutic benefit in children with dilated cardiomyopathy (DCM). We aimed to determine the safety and efficacy of CDCs in a porcine model of DCM and translate the preclinical results into this patient population. A swine model of DCM using intracoronary injection of microspheres created cardiac dysfunction. Forty pigs were randomized as preclinical validation of the delivery method and CDC doses, and CDC-secreted exosome (CDCex)-mediated cardiac repair was analyzed. A phase 1 safety cohort enrolled five pediatric patients with DCM and reduced ejection fraction to receive CDC infusion. The primary endpoint was to assess safety, and the secondary outcome measure was change in cardiac function. Improved cardiac function and reduced myocardial fibrosis were noted in animals treated with CDCs compared with placebo. These functional benefits were mediated via CDCex that were highly enriched with proangiogenic and cardioprotective microRNAs (miRNAs), whereas isolated CDCex did not recapitulate these reparative effects. One-year follow-up of safety lead-in stage was completed with favorable profile and preliminary efficacy outcomes. Increased CDCex-derived miR-146a-5p expression was associated with the reduction in myocardial fibrosis via suppression of proinflammatory cytokines and transcripts. Collectively, intracoronary CDC administration is safe and improves cardiac function through CDCex in a porcine model of DCM. The safety lead-in results in patients provide a translational framework for further studies of randomized trials and CDCex-derived miRNAs as potential paracrine mediators underlying this therapeutic strategy.

DOI： 10.1126/scitranslmed.abb3336

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：W.B. Saunders  

The Norwood procedure consists of a complex reconstruction resulting in: (1) unobstructed systemic outflow from the right ventricle to neo-aorta, (2) creation of large atrial septal defect, and (3) a controlled source of pulmonary blood flow (PBF) from the systemic circulation. The Sano modification provides the source of PBF from the right ventricle. Both procedures have advantages and disadvantages, therefore, there have been many debates regarding which procedure is better as a first stage palliation. Another ongoing debate of first stage palliation is the method of reconstruction of the neo-aorta. Aortic arch obstruction increases afterload and leads to ventricular dysfunction and tricuspid regurgitation. Recent SVR Trial identified recurrent coarctation requiring intervention in 18% of patients. We believe that another important point regarding reconstruction of the neo-aorta is the use of only native tissue and minimizing the use of inelastic patch material. Because elastic properties of the arterial tree are known to have a profound influence on ventricular function, arterial elasticity may therefore be of particular importance to those patients with hypoplastic left heart syndrome. During the past 20 years, we have modified our original technique of aortic arch reconstruction. Proximal arch plication and creation of elongated flaps of the main pulmonary artery are 2 of our recent modifications which facilitate neo-aortic reconstruction with native tissue. We describe a technique where direct anastomosis of neo-aorta is achieved with avoidance of foreign material in a majority of our patients.

DOI： 10.1053/j.optechstcvs.2020.07.004

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DOI： 10.1016/j.case.2020.08.002

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Language：Japanese   Publishing type：Research paper (scientific journal)  

A large population of patients with congenital heart disease with untreated systemic to pulmonary shunts( left to right shunts) will develop pulmonary arterial hypertension( PAH). There are 2 different statuses of an increase in pulmonary arterial pressure. One is high resistance due to high pulmonary blood flow (high flow with high resistance), another one is low pulmonary flow due to high resistance (low flow with high resistance). Chronic large left-to-right shunt induced severe pulmonary vascular disease and pulmonary hypertension. This was then subsequence of low pulmonary blood flow with high pulmonary vascular resistance. We have to avoid this situation and have to do intervention within the pulmonary vascular reactivity has been left. For this reason, preoperative treatment for avoidance of high flow, appropriate timing of interventions and postoperative various managements are important factors as aiming of low pulmonary resistance in this group. Recent advances in PAH-specific drugs have dramatically changed the therapeutic strategy for PAH. A strategy that includes "treatment" with PAH-specific drugs initially and then "repair" by closure of the cardiac defect (i.e. "treat and repair") was devised, and has been attempted, in patients with PAH associated with a cardiac defect.

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It has been controversial how to manage severe dysfunction of the systemic right ventricle and severe tricuspid regurgitation for adult patients with congenitally corrected transposition of the great arteries. We reported a 43-year-old man with these disorders, who received pulmonary artery banding and cardiac resynchronization therapy without cardiopulmonary bypass. The degree of the tricuspid regurgitation was improved to mild-moderate and systemic right ventricular ejection fraction was improved from 28 to 45% after surgery. Cardiopulmonary exercise testing showed that the predictive value of anaerobic threshold and peak oxygen uptake improved from 65 to 99% and 59 to 92%, respectively. In conclusion, pulmonary artery banding and cardiac resynchronization therapy can be the first choice of surgery for severe tricuspid regurgitation with severely impaired systemic right ventricular function. After improving systemic right ventricular dysfunction tricuspid surgery could be the next choice as a surgical intervention in the future.

DOI： 10.1007/s11748-019-01171-x

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OBJECTIVES: The aim of this study was to evaluate the long-term outcomes of the Norwood procedure with right ventricle-pulmonary artery (RV-PA) conduit for hypoplastic left heart complex. METHODS: A retrospective observational study was performed in 136 patients with hypoplastic left heart complex who underwent a Norwood procedure with RV-PA conduit between 1998 and 2017. The probabilities of survival, reintervention and Fontan completion were analysed. RESULTS: Stage 1 survival was 91.9% (125/136). Reintervention for PA stenosis was needed for 22% and 30% at stages 2 and 3, respectively, while 15% underwent reintervention for aortic arch recoarctation. Among 106 bidirectional Glenn survivors, 93 (68% of the total number of patients) had a Fontan completion, while 4 were not considered to be Fontan candidates. Risk factors for overall mortality included weighing <2.5 kg at the time of the Norwood procedure, intact atrium septum, total anomalous pulmonary vein connection and more than mild atrioventricular regurgitation at the time of the Norwood procedure. Overall survival was 80.9%, 72.3% and 62.8% at 1, 5 and 20 years, respectively. CONCLUSIONS: Probabilities of survival and Fontan completion were acceptable under the current surgical strategy incorporating RV-PA Norwood procedure as the first palliation. Incorporating a strategy to maintain PA growth and ventricular function through the staged repair is of prime importance. Further studies are necessary to observe changes in atrioventricular regurgitation as well as in right ventricular function, in patients who require atrioventricular valve interventions during the staged Fontan completion.

DOI： 10.1093/ejcts/ezaa041

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BACKGROUND: Previous studies demonstrated that the incidence of atrial fibrillation (AF) was significantly increased when patients aged ≥40 years had surgical atrial septal defect (ASD) closure (sASD). However, limited information is available on such findings in transcatheter ASD closure (tASD). The purpose of this study was to investigate the incidence of newly developed AF after tASD in patients aged ≥40 years in whom preoperative AF or atrial flutter (AFL) had not been detected and to compare with the incidence after sASD. METHODS: The medical records of patients aged ≥40 years without a history of AF or AFL who underwent tASD (n = 281) or sASD (n = 24) were reviewed. Patients who had catheter ablation before the ASD closure were excluded. Patients with a patent foramen ovale were also excluded. The incidence of newly developed AF after ASD closure and the risk factors for that were evaluated statistically. RESULTS: Eleven patients had newly developed AF (5 in tASD and 6 in sASD) postoperatively including 6 persistent AF (3 each after tASD and sASD). The cumulative incidence of newly developed AF was 0.7% in tASD and 16.7% in sASD, and 2.7% and 20.8% at 1 and 5 years, respectively (p < 0.001). ASD diameter ≥30 mm and sASD were potential risk factors for newly developed AF after ASD closure and postoperative persistent AF. CONCLUSIONS: In patients aged ≥40 years without a history of AF or AFL, the incidence of newly developed AF after tASD closure was lower than that after sASD. A large ASD more than 30 mm diameter was a potential risk factor for development of AF even if it is closed by transcatheter procedure. Further long-term evaluation after tASD is required to clarify preventive benefit for new onset AF in adult ASD population.

DOI： 10.1016/j.jjcc.2020.01.008

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：W.B. Saunders  

An increasing number of patients will require pulmonary valve replacement following repair of tetralogy of Fallot. Most of these patients have a history of surgical repair with a transannular patch (ie, pulmonary valve not preserved). Ten to twenty years after the initial repair, severe pulmonary regurgitation results in right ventricular dilation and dysfunction. There are multiple prosthetic valve options and techniques to use when performing pulmonary valve replacements. Herein, we describe our technique of bioprosthetic pulmonary valve replacement.

DOI： 10.1053/j.optechstcvs.2020.01.001

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DOI： 10.1007/s10396-019-00986-8

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：TECH SCIENCE PRESS  

Background: There is limited information on outcomes of adult patients with pulmonary atresia with intact ventricular septum (PA-IVS) due to the low incidence of disease and the large variation of surgical histories. Methods: Among 58 patients with repaired PA-IVS, a total of 32 patients aged >= 16 years and who were followed at our institution between January 2003 and December 2018 were reviewed. Surgical history, clinical outcomes, and laboratory, echocardiographic and electrocardiographic data were obtained by chart review. Results: Follow-up was from the age of 16 years and the median age at the latest follow-up was 23.7 years. Twenty-four patients had undergone biventricular repair (BVR), 3 had undergone one-and-a half ventricular repair (1.5VR), and 5 had undergone univentricular repair. Over a median follow-up period of 7.7 years (interquartile range: 4.1-11.0 years), 1 BVR patient died suddenly and 7 patients had heart failure. Arrhythmias were present in 5 patients. Ten patients underwent surgical re-interventions, including 4 BVR take-downs with conversion to 1.5VR and 3 Fontan conversions. Overall survival, heart failure-free, arrhythmia-free, and surgical re-intervention-free rates at 5 years and 10 years from the age of 16 years were 96.2% (95% confidence interval [CI], 77.2-99.4) and 96.2% (95% CI, 77.2-99.4), 81.4% (95% CI, 62.1-92.1) and 74.6% (95%CI, 52.3-88.7), 88.7% (95% CI, 70.1-96.3) and 75.9% (95% CI, 51.7-90.2), and 80.7% (95% CI, 60.8-91.8) and 70.8% (95% CI, 49.7-85.7), respectively. Conclusion: Adults with PA-IVS have preserved long-term survival regardless of the early operative strategy, while they are at risk for heart failure, arrhythmia, and surgical re-intervention. Thus, detailed and continued follow-up is mandatory for all PA-IVS patients from childhood to adulthood.

DOI： 10.32604/CHD.2020.011579

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Language：Japanese   Publisher：(一社)日本外科学会  

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Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Pulmonary vein stenosis (PVS) is a condition with challenging treatment and leads to severe cardiac failure and pulmonary hypertension. Despite aggressive surgical or catheter-based intervention, the prognosis of PVS is unsatisfactory. This study aimed to assess the prognosis and to establish appropriate treatment strategies. METHODS: We retrospectively reviewed endovascular treatments for PVS (2001-2017) from the clinical database at the Okayama University Hospital. RESULTS: A total of 24 patients underwent PVS associated with total anomalous pulmonary venous connection and 7 patients underwent isolated congenital PVS. In total, 53 stenotic pulmonary veins were subjected to endovascular treatments; 40 of them were stented by hybrid (29) and percutaneous procedures (11) (bare-metal stent, n = 34; drug-eluting stent, n = 9). Stent size of hybrid stenting was larger than percutaneous stenting. Median follow-up duration from the onset of PVS was 24 months (4-134 months). Survival rate was 71 and 49% at 1 and 5 years, respectively. There was no statistically significant difference between stent placement and survival; however, patients who underwent bare-metal stent implantation had statistically better survival than those who underwent drug-eluting stent implantation or balloon angioplasty. Early onset of stenosis, timing of stenting, and small vessel diameter of pulmonary vein before stenting were considered as risk factors for in-stent restenosis. Freedom from re-intervention was 50 and 26% at 1 and 2 years. CONCLUSIONS: To improve survival and stent patency, implantation of large stent is important. However, re-intervention after stenting is also significant to obtain good outcome.

DOI： 10.1017/S1047951119001495

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Background: The increase in the use of cardiac implantable electronic devices (CIEDs) has been associated with an increase in CIED-related infections. Transvenous lead extraction is safe and effective for patients with CIED-related infections; however, the mortality rate in these patients is high. The prognosis after transvenous lead extraction in Japanese patients, especially those with lead-related infective endocarditis, has not been evaluated. Then, the purpose of this study is to clarify the prognosis after transvenous lead extraction in Japanese patients with CIED-related infections at a single Japanese center. Methods: A total of 107 patients who underwent transvenous lead extraction were retrospectively reviewed. The patients were divided into a lead-related infective endocarditis group (n = 32) and a pocket infection group (n = 75). Procedure success rate and prognosis after lead extraction were evaluated between the two groups. Results: Procedure success rate was not significantly different between the groups. There were no deaths associated with the procedure or with infection. The survival rate was not significantly different at 1 year or at a median of 816 days (lead-related infective endocarditis vs pocket infection; 93.7% vs 94.7%, P = 1.000; 78.1% vs 81.3%, P = 0.791) Time to reimplantation and duration of hospital stay and antibiotics therapy were significantly longer for patients with lead-related infective endocarditis. Conclusion: In this study, the prognosis for patients with lead-related infective endocarditis after transvenous lead extraction was favorable. Thus, extraction should be strongly recommended, even if the general condition of the patient is poor.

DOI： 10.1002/joa3.12164

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OBJECTIVES: The role of intraoperative cardiopulmonary bypass (CPB) in lung transplant (LTx) surgery is controversial. CPB enables slow pulmonary reperfusion and initial ventilation with low oxygen concentrations, both theoretically protective of transplanted lungs. In this study, we explored clinical outcomes following extended criteria donor LTx surgery implementing a thoroughly protective allograft reperfusion strategy using CPB. METHODS: Thirty-nine consecutive adult patients who underwent bilateral LTx with elective CPB and protective allograft reperfusion were reviewed. Bilaterally implanted lungs were reperfused simultaneously, via slow CPB flow reduction and initial ventilation with 21% oxygen and nitric oxide, followed by a brief modified ultrafiltration. During weaning from CPB, mean pulmonary arterial pressure was strictly maintained at 10-15 mmHg by controlling CPB and pulmonary flow. The clinical outcomes in 23 patients who received lungs from extended criteria donors (ECD group) were elucidated and compared to 16 patients undergoing LTx from standard criteria donors (SCD group). RESULTS: No life-threatening deterioration was observed to graft functionality during the first 72 h after LTx in the ECD group; however, only one patient required post-transplant extracorporeal membrane oxygenation. In three of 23 ECD LTx patients (12%), surgical revision for bleeding was required. Survival outcomes for the ECD group were favorable, with 100% survival at 6-months, 87.0% at 1-year, and 80.7% at 5-years. Outcomes in the ECD group were comparable to those in the SCD group. CONCLUSIONS: Despite a certain extent of risk associated with full-dose heparinization, use of CPB does not undermine survival outcomes after ECD LTx surgery if protective allograft reperfusion is securely performed.

DOI： 10.1007/s11748-019-01067-w

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The majority of marathon deaths are caused by sudden cardiac arrest (SCA), which occur in approximately 1 in 57,000 runners. Such deaths are more common among older males and usually occur in the last 4 miles of the racecourse. Although prompt resuscitation, including early use of an automated external defibrillator (AED), improves survival, the deployment of enough trained medical staff and AEDs is difficult due to increased cost. Moreover, most victims of exercise-related SCA have no premonitory symptoms. Therefore, we tried to use a novel approach to prevent sudden cardiac deaths (SCD) related to SCA using real-time electrocardiographic tele-monitoring system, as an initial trial to assess operative possibility in a full marathon. As a result, 3 out of 5 runners had reasonable measurement results and sufficient tele-monitoring without complications related to this trial was possible. However, many investigations and improvements, such as improving cost-effectiveness, reducing noise, and automating the monitoring system, are needed for practical application of these devices for athletes. As a next step, we would establish a novel strategy to reduce SCDs in athletes using next-generation devices, which include an alarm system associated with early application of AED. <Learning objectives: Sudden cardiac arrest (SCA) is a major problem in sports cardiology. Here we investigated a novel approach using a real-time tele-monitoring system of electrocardiogram (ECG) to prevent sudden cardiac deaths by making use of an advanced alarm system which responds to SCA risk. Three out of five cases we monitored showed reasonable measurement of ECG with centralized observation in full marathon. This is the first report of this method, which may lead to the effective application of automated external defibrillator in athletes.>.

DOI： 10.1016/j.jccase.2019.03.008

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DOI： 10.1177/0218492318811557

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Background:
Chronic angiotensin II (AngII) infusion promotes ascending aortic dilation in C57BL/6J mice. Meanwhile, vasohibin-2 (VASH2) is an angiogenesis promoter in neovascularization under various pathologic conditions. The aim of this study was to investigate whether exogenous VASH2 influences chronic AngII-induced ascending aortic dilation.
Methods and Results:
Eight-ten-week-old male C57BL/6J mice were injected with adenovirus (Ad) expressing either VASH2 or LacZ. One week after the injection, mice were infused with either AngII or saline s.c. for 3 weeks. Mice were divided into 4 groups: AngII+VASH2, AngII+LacZ, saline+VASH2, and saline+LacZ. Overexpression of VASH2 significantly increased AngII-induced intimal areas as well as the external diameter of the ascending aorta. In addition, VASH2 overexpression promoted ascending aortic medial elastin fragmentation in AngII-infused mice, which was associated with increased matrix metalloproteinase activity and medial smooth muscle cell (SMC) apoptosis. On western blot analysis, accumulation of apoptotic signaling proteins, p21 and p53 was increased in the AngII+VASH2 group. Furthermore, transfection of human aortic SMC with Ad VASH2 increased p21 and p53 protein abundance upon AngII stimulation. Positive TUNEL staining was also detected in the same group of the human aortic SMC.
Conclusions:
Exogenous VASH2 exacerbates AngII-induced ascending aortic dilation in vivo, which is associated with increased medial apoptosis and elastin fragmentation.

DOI： 10.1253/circrep.CR-19-0008

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Language：Japanese   Publishing type：Research paper (scientific journal)  

Ebstein's anomaly (EA) is a rare congenital cardiac malformation and this anomaly is a disorder of a tricuspid valve development in which the valve leaflets fail to delaminate properly from the ventricular wall. Clinical symptoms are age dependent and include cyanosis (size of inter-atrial communication), right-sided heart failure, arrhythmias, and general fatigue on exercise. Optimal timing of surgical intervention is often difficult and must be individualized. Patients have good long-term survival and functional outcomes after undergoing surgery for Ebstein's anomaly in adult population. Operation includes tricuspid valve repair or replacement, closure of any inter-atrial communications, and appropriate antiarrhythmia procedures.

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BACKGROUND: Rashkind balloon atrial septostomy (BAS) can be challenging in infants with hypoplastic left heart syndrome (HLHS) and small atrial septal defect (ASD). METHODS: We retrospectively reviewed all infants with HLHS who underwent surgery and BAS between January 2006 and December 2015. The infants were divided into three groups: no BAS; catheter BAS; and open AS. Infants who underwent catheter BAS were divided into two groups based on atrial septal anatomy: standard and complex. RESULTS: Of the 70 patients, 57 (81%) underwent Glenn surgery. Subsequently, a significant difference in survival was observed: 86% (44/51), 91% (10/11), and 25% (2/8) in the no BAS, catheter BAS, and open AS groups, respectively (P = 0.0002). No significant difference was seen between the no BAS and the catheter BAS groups (P = 1.0). In the 56 patients who underwent catheterization after surgery, no intergroup differences in mean pulmonary artery pressure, pulmonary vascular resistance, or pulmonary artery index were found. We classified catheter BAS into standard (n = 5) and complex (n = 5) based on ASD location, and septum thickness. All patients in the standard group underwent complete Rashkind BAS, but in the complex group, only one patient underwent complete Rashkind BAS, with the remaining requiring initial static BAS (P = 0.048). Following septostomy, ASD size, ASD flow, and percutaneous oxygen saturation (SpO2 ) were not significantly different between the two groups. CONCLUSIONS: Catheter BAS is effective in infants with HLHS and a restrictive atrial septum. Infants with standard or complex atrial septum can achieve equivalent outcomes despite more patients often requiring static BAS.

DOI： 10.1111/ped.13716

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Language：Japanese   Publisher：日本臨床麻酔学会  

小児におけるECMO(Extracorporeal Membrane Oxygenation)治療は、先天性心疾患に対しても重要な適応がある。小児複雑心奇形周術期におけるECMO導入のコンセンサスの得られた明確な基準はないが、循環破綻による多臓器障害を合併した状態での導入より、計画的早期の導入の方が離脱率の向上につながると考えられる。出血性合併症が問題となりやすいが、病態を把握し補充療法に努める。心機能の回復には心室の十分な減圧が必要である。小児複雑心奇形の特徴的な疾患である単心室症例のECMO管理では循環生理および解剖学的特徴をよく理解し、各々の状態に応じた管理が肝要である。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J01620&link_issn=&doc_id=20190319280006&doc_link_id=%2Fci4jjsca%2F2019%2F003902%2F007%2F0148-0154%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fci4jjsca%2F2019%2F003902%2F007%2F0148-0154%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：English   Publishing type：Research paper (scientific journal)   Publisher：SAGE Publications Ltd  

Pulmonary artery aneurysms (PAA) can be complicated with pulmonary arterial hypertension (PAH), causing sudden death due to PA rupture and dissection. Because treatment with PAH-targeted drugs does not always prevent PAA progression, prophylactic surgical repair of the PAA seems a promising alternative. However, although it avoids rupture and dissection of the PAs, additional benefits have not been forthcoming. We therefore present two patients with co-existing PAH and a PAA who underwent surgical repair of the aneurysm. Following the surgery, their lung function and pulmonary hypertension improved. Optimal treatment of PAA remains uncertain, however, with no clear guidelines regarding the best therapeutic approach. This case series provides physicians with reasons to repair PAA surgically in patients with PAH.

DOI： 10.1177/2045894019831217

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DOI： 10.1016/j.hrcr.2018.10.008

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AIM: Since 1998, the leading cause of chronic hemodialysis in Japan has been diabetic nephropathy. Diabetes mellitus is known to be a risk factor for frailty, but it still remains unknown whether diabetic nephropathy is associated with frailty in chronic dialysis patients. The authors carried out the present study to reveal the association between frailty and diabetic nephropathy in chronic hemodialysis patients. METHODS: A total of 355 patients who were on hemodialysis were recruited. Participants were divided into two groups of either patients who suffered diabetic nephropathy with end-stage renal disease (DN group, n = 150) or not (Non-DN group, n = 205). The authors investigated the difference of the prevalence of frailty between the two groups. Furthermore, the authors examined the risk factors for frailty. RESULTS: The prevalence of frailty in the DN group was significantly higher than that in the Non-DN group (28.0% vs 16.5%, P = 0.0161). To evaluate the association between frailty and its risk factors, we compared frail patients (n = 71) and non-frail patients (n = 262). After adjusting their interrelationships by using multivariate logistic regression analysis, diabetic nephropathy was determined as a significant risk factor for frailty. CONCLUSIONS: The authors found the close association between frailty and diabetic nephropathy in chronic hemodialysis patients. Geriatr Gerontol Int 2018; 18: 1597-1602.

DOI： 10.1111/ggi.13534

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DOI： 10.1016/j.jcin.2018.06.055

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Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

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Objectives The clinical condition of frailty is a common problem in the elderly population. However, the relationship between peripheral artery disease and frailty in hemodialysis patients remains unknown. The aim of this study was to identify the relationships between peripheral artery disease and frailty in Japanese chronic hemodialysis patients. Methods A total of 362 chronic hemodialysis patients who regularly visited six institutions were enrolled. To evaluate frailty, the modified Fried's frailty phenotype adjusted for Japanese were used. Peripheral artery disease was defined as ankle-brachial index <0.9. Results Of 362 patients, 62 patients (17.1%) were categorized as peripheral artery disease group and 300 patients (82.9%) as Non-peripheral artery disease group. The prevalence of frailty in the peripheral artery disease group was significantly higher than in the Non-peripheral artery disease group (34% vs. 18%, P = 0.0103). Non-shunt side grip strength was significantly stronger in the Non-peripheral artery disease group (23.6 kg vs. 17.0 kg, P < 0.0001). Thigh circumferences were also significantly larger in the Non-peripheral artery disease group (41.7 cm vs. 39.7 cm, P = 0.0054). A multivariate logistic regression analysis demonstrated that the factors independently associated with peripheral artery disease were as follows: frailty (odds ratio = 2.06, 95% confidence interval 1.09-3.89) and myocardial infarction (odds ratio = 3.74, 95% confidence interval 2.05-6.83). Conclusions It is concluded that peripheral artery disease is closely associated with frailty in hemodialysis patients.

DOI： 10.1177/1708538118756690

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Introduction: Recent advances in pulmonary arterial hypertension (PAH)-specific drugs have dramatically changed the therapeutic strategy for PAH. A strategy that includes 'treatment' with PAH-specific drugs initially and then 'repair' by closure of the cardiac defect (i.e. 'treat and repair') was devised, and has been attempted, in patients with PAH associated with a cardiac defect. Case presentation: We present three cases of severe PAH associated with a ventricular septal defect (VSD) in adult patients who were initially treated with PAH-specific drugs followed by VSD closure. Two of the patients were treated with a combination of an endothelin receptor antagonist (ERA), phosphodiesterase type 5 inhibitor, and intravenous prostacyclin before VSD closure. The third patient was treated with an ERA and pulmonary artery banding before VSD closure. After 12 months of anti-PAH treatment, the pulmonary vascular resistance index and the ratio of the pulmonary vascular index to the systemic vascular resistance index decreased to levels that allowed VSD closure. At the mid- and long-term follow-up measurements after surgical closure of the VSD, the mean pulmonary artery pressure had markedly decreased. Discussion: Our case series suggests that the treat-and-repair strategy is a promising approach for adult patients with severe PAH associated with VSD.

DOI： 10.1093/ehjcr/yty033

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Language：Japanese   Publisher：(NPO)日本血管外科学会  

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BACKGROUND: Pulmonary artery (PA) dilatation is usually observed in patients with pulmonary hypertension (PH), but a PA aneurysm (PA diameter > 40mm) is rare. The difference between characteristics of patients with and those without progression of PA diameter remains poorly understood. We assessed the changes in PA diameter in patients with PH coexisting with and without a PA aneurysm. METHODS: We investigated the changes in PA diameter by multi-detector computed tomography performed twice with an interval of more than one year in 44 patients with PH. Seventeen patients had a PA aneurysm and 27 patients did not have a PA aneurysm at baseline. RESULTS: The median follow-up period was 3.6 years. All patients received medical or invasive treatment for PH. At baseline, main PA diameters were 52±15mm in patients with a PA aneurysm and 33±3mm in patients without a PA aneurysm. Mean PA pressure was higher in patients with a PA aneurysm than in those without a PA aneurysm (61±15mmHg vs. 51±16mmHg, p=0.04). At follow-up, mean PA pressure significantly decreased in both patients with a PA aneurysm (44±11mmHg) and patients without a PA aneurysm (41±18mmHg). Main PA diameter significantly increased in patients with a PA aneurysm (65±28mm, change ratio: 23.3%), while it did not increase in patients without a PA aneurysm (32±3mm, change ratio: -3.1%). CONCLUSIONS: PA dilatation progressed in patients with a PA aneurysm despite treatment of PH. The progression of PA dilatation is independent of reduction of PA pressure by PH treatment.

DOI： 10.1016/j.jjcc.2017.11.005

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Left ventricular (LV) rupture after myocardial infarction (MI) occasionally results in formation of LV pseudoaneurysm (LVPA) which is prone to rupture because of its thin wall. However, cases of LVPA without ST changes including segment elevation in electrocardiogram (ECG) are rare. In this case, we describe a patient who had relatively mild symptoms and giant LVPA with no specific ECG changes following MI with a confirmed diagnosis via transthoracic echocardiography. Although surgical treatment options are often recommended, conservative therapy was adopted, following which the patient had been well-medicated using antihypertensive drugs and anticoagulants. <Leaning objectives: Left ventricular pseudoaneurysm (LVPA) is usually accompanied by ST segment changes on electrocardiogram (ECG) due to myocardial damage. However, we should take into account a LVPA without ECG specific changes, so echocardiography is better to be considered for an identification. Although many LVPA patients undergo surgery because of risk for rupture, some cases with stable hemodynamic status can have long-term survival with conservative therapy such as anti-hypertension and coagulation.>.

DOI： 10.1016/j.jccase.2018.01.006

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

ベーチェット病を基礎疾患に持つ患者の大動脈弁手術は、術後高率に弁周囲逆流、弁動揺、そして吻合部仮性瘤を合併することが知られている。患者は37歳の男性で、大動脈弁置換術直後に高度な弁周囲逆流により基部置換術が行われ、その後も吻合部仮性瘤を繰り返し、経過中に不全型ベーチェット病と診断されステロイドによる加療が開始された。その後、ベーチェット病に対する免疫抑制療法導入中にStaphylococcus epidermidisによる菌血症をきたし、大動脈基部に吻合部仮性瘤が再燃した。6週間の抗生剤加療の後にホモグラフトによる大動脈基部置換術を施行し5年間、問題なく経過しているため報告する。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2018&ichushi_jid=J01122&link_issn=&doc_id=20180531170009&doc_link_id=10.4326%2Fjjcvs.47.133&url=https%3A%2F%2Fdoi.org%2F10.4326%2Fjjcvs.47.133&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

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RATIONALE: Intracoronary administration of cardiosphere-derived cells (CDCs) in patients with single ventricles resulted in a short-term improvement in cardiac function. OBJECTIVE: To test the hypothesis that CDC infusion is associated with improved cardiac function and reduced mortality in patients with heart failure. METHODS AND RESULTS: We evaluated the effectiveness of CDCs using an integrated cohort study in 101 patients with single ventricles, including 41 patients who received CDC infusion and 60 controls treated with staged palliation alone. Heart failure with preserved ejection fraction (EF) or reduced EF was stratified by the cardiac function after surgical reconstruction. The main outcome measure was to evaluate the magnitude of improvement in cardiac function and all-cause mortality at 2 years. Animal studies were conducted to clarify the underlying mechanisms of heart failure with preserved EF and heart failure with reduced EF phenotypes. At 2 years, CDC infusion increased ventricular function (stage 2: +8.4±10.0% versus +1.6±6.4%, P=0.03; stage 3: +7.9±7.5% versus -1.1±5.5%, P<0.001) compared with controls. In all available follow-up data, survival did not differ between the 2 groups (log-rank P=0.225), whereas overall patients treated by CDCs had lower incidences of late failure (P=0.022), adverse events (P=0.013), and catheter intervention (P=0.005) compared with controls. CDC infusion was associated with a lower risk of adverse events (hazard ratio, 0.411; 95% CI, 0.179-0.942; P=0.036). Notably, CDC infusion reduced mortality (P=0.038) and late complications (P<0.05) in patients with heart failure with reduced EF but not with heart failure with preserved EF. CDC-treated rats significantly reversed myocardial fibrosis with differential collagen deposition and inflammatory responses between the heart failure phenotypes. CONCLUSIONS: CDC administration in patients with single ventricles showed favorable effects on ventricular function and was associated with reduced late complications except for all-cause mortality after staged procedures. Patients with heart failure with reduced EF but not heart failure with preserved EF treated by CDCs resulted in significant improvement in clinical outcome. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifiers: NCT01273857 and NCT01829750.

DOI： 10.1161/CIRCRESAHA.117.312311

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：W.B. Saunders  

It has become increasingly evident that anomalous aortic origin of a coronary artery is considered as an important anatomic anomaly in adolescents and adults. The question is whether or not surgical repair should be performed in asymptomatic patients. Previous reports showed that the numbers of asymptomatic patients who result in a sudden cardiac death are not negligible. Another reason to proceed to surgical intervention is the improvement in understanding this unique anatomy and the refinement of surgical techniques that brought the satisfied outcomes. Physiologic repair, that is, coronary artery bypass grafting was used to be the first choice of surgery
however, anatomic repair, such as coronary implantation and repair of intramural coronary artery is considered favorable in adolescents as “radical” oppose to “palliative” repair. We report our technique to relieve the intramural coronary artery based on the experience in arterial switch operation performed in neonates.

DOI： 10.1053/j.optechstcvs.2018.10.002

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A 75-year-old woman presented with severe tricuspid regurgitation requiring surgical repair. She had extremely rare anomalies in systemic venous return in spite of situs solitus: persistent left superior vena cava with absent right superior vena cava, infrahepatic inferior vena cava interruption, and hemiazygos continuation to a persistent left superior vena cava. These unusual systemic venous anomalies had significantly enlarged the coronary sinus, and the stretched coronary sinus orifice changed the geometry of the tricuspid annulus and triggered tricuspid regurgitation. There were technical difficulties in establishing cardiopulmonary bypass and achieving successful tricuspid valve repair.

DOI： 10.1177/0218492318759153

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Language：Japanese   Publisher：(一社)日本循環器学会  

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DOI： 10.24105/ejbi.2018.14.1.9

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DOI： 10.1016/j.jtcvs.2017.09.018

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Language：Japanese   Publisher：(一社)日本集中治療医学会  

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DOI： 10.24509/jpccs.180206

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

背景:左心低形成症候群(HLHS)では左房容積が小さく、心房中隔の形態や心房中隔欠損(ASD)の大きさによってRashkind Balloon Atrial Septostomy(BAS)を行うことが困難な場合もある。目的:当院におけるHLHSに対するBASの有効性について検討した。対象と方法:2006年1月から2015年12月までの10年間にHLHSに対してカテーテル治療・手術を施行した患者におけるBASの有効性について検討した。またcatheter BASを施行した群についてASDの形態別に分けて有効性について両群比較検討した。結果:全HLHS70例中のGlenn手術到達は57例(81%)であり、BAS未施行群では86%(44/51)、catheter BAS群では91%(10/11)、open BAS群では25%(2/8)と統計学的有意差を認めたが(p=0.0002)、BAS未施行群とcatheter BAS群では同等の結果であった。(p=1.0)Glenn手術後にカテーテル検査を施行した56例について検討すると、BAS未施行群、catheter BAS施行群、open BAS施行群の平均肺動脈圧、肺血管抵抗、PAIいずれも3群間に差は認めなかった。次にCatheter BAS施行群をASDの位置・大きさ・atrial septumの厚さからstandard ASD(n=5)、complex ASD(n=5)に分類し、ASDの形態別にBAS施行方法を検討すると、standard ASD群では全例Rashkind BAS単独施行で効果を得たが、complex ASD群ではRashkind BAS単独施行症例は1例のみで、Static BASを先行させRashkind BASに到達した症例が4例であった(p=0.048)。BAS後のASD size、ASD flow、SpO2は2群間で統計学的有意差を認めなかった。結論:catheter BASは有効で、Glenn後のカテーテルデータではBAS未施行群と有意差は認めなかった。またcomplex ASD群の場合には、Static BASを先行し、Rashkind BASを追加することで、standard ASD群と同等の効果を得ることができた。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J02003&link_issn=&doc_id=20180418180002&doc_link_id=%2Fee7shoni%2F2017%2F003306%2F003%2F0423-0430%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fee7shoni%2F2017%2F003306%2F003%2F0423-0430%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

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DOI： 10.1093/eurheartj/ehx128

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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RATIONALE: Patients with single ventricle physiology are at high risk of mortality resulting from ventricular dysfunction. The preliminary results of the phase 1 trial showed that cardiosphere-derived cells (CDCs) may be effective against congenital heart failure. OBJECTIVE: To determine whether intracoronary delivery of autologous CDCs improves cardiac function in patients with single ventricle physiology. METHODS AND RESULTS: We conducted a phase 2 randomized controlled study to assign in a 1:1 ratio 41 patients who had single ventricle physiology undergoing stage 2 or 3 palliation to receive intracoronary infusion of CDCs 4 to 9 weeks after surgery or staged reconstruction alone (study A). The primary outcome measure was to assess improvement in cardiac function at 3-month follow-up. Four months after palliation, controls had an alternative option to receive late CDC infusion on request (study B). Secondary outcomes included ventricular function, heart failure status, somatic growth, and health-related quality of life after a 12-month observation. At 3 months, the absolute changes in ventricular function were significantly greater in the CDC-treated group than in the controls (+6.4% [SD, 5.5] versus +1.3% [SD, 3.7]; P=0.003). In study B, a late CDC infusion in 17 controls increased the ventricular function at 3 months compared with that at baseline (38.8% [SD, 7.7] versus 34.8% [SD, 7.4]; P<0.0001). At 1 year, overall CDC infusion was associated with improved ventricular function (41.4% [SD, 6.6] versus 35.0% [SD, 8.2]; P<0.0001) and volumes (P<0.001), somatic growth (P<0.0001) with increased trophic factors production, such as insulin-like growth factor-1 and hepatocyte growth factor, and quality of life, along with a reduced heart failure status (P<0.0001) and cardiac fibrosis (P=0.014) relative to baseline. CONCLUSIONS: Intracoronary infusion of CDCs after staged palliation favorably affected cardiac function by reverse remodeling in patients with single ventricle physiology. This impact may improve heart failure status, somatic growth, and quality of life in patients and reduce parenting stress for their families. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01829750.

DOI： 10.1161/CIRCRESAHA.116.310253

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Language：Japanese   Publisher：(一社)日本循環器学会  

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DOI： 10.1161/CIRCIMAGING.116.005839

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Language：Japanese   Publisher：Japanese Society of Pediatric Cardiology and Cardiac Surgery  

<p>Background: Protein-losing enteropathy (PLE) following the Fontan operation has been an extremely difficult complication to treat successfully. This study aimed to assess the prognosis and to establish appropriate treatment strategies for PLE.</p><p>Methods: From 1991 to 2014, 23 patients with PLE following the Fontan operation were identified from clinical database at Okayama University Hospital. Data were collected retrospectively.</p><p>Results: A total of 401 patients underwent Fontan operation at our institution during the study period. In this cohort, the 5.7％ incidence of PLE was relative to the total number of Fontan operations. Following PLE diagnosis, patients had a 68％ and 54％ survival rate at 5 and 10 years, respectively. The causes of death included sepsis, multiple organ failure, heart failure, and intracranial bleeding. In the treatment of PLE, 23 (100％) patients were treated with medication, 10 (43％) patients underwent catheter intervention and 15 (65％) patients underwent surgery. Patients who died were older at the time of PLE diagnosis and were associated with a longer interval between the Fontan operation and PLE diagnosis. Decreased survival was observed in patients with the following conditions post-PLE treatment: a cardiac index (CI)＜2.8 L/min/mm, decreased ventricular ejection fraction (vEF)＜50％, and high central venous pressure (meanCVP)≧15 mmHg.</p><p>Conclusion: Our current treatment for PLE was only partially successful. Strategies for reducing CVP and improving both CI and vEF may be effective. Further studies are necessary to determine better treatment strategies for the future.</p>

DOI： 10.9794/jspccs.33.202

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OBJECTIVES: To achieve the growth of right-sided heart structures, our choice of the first palliation for patients with pulmonary atresia and intact ventricular septum (PA-IVS) includes a modified Blalock-Taussig shunt (BTS) with pulmonary valvotomy. We sought to analyse the impact of the first palliation on the growth of right-sided heart structures and factors associated with a choice of definitive surgical procedure. METHODS: Fifty patients with PA-IVS who underwent a staged surgical approach from 1991 to 2012 were retrospectively reviewed. RESULTS: Right ventricular (RV)-coronary artery fistulas were seen in 42% of patients at the time of birth. All 50 patients had a modified BTS with pulmonary valvotomy. Six patients died after the first palliation or inter-stage. Thirty patients achieved a biventricular repair (BVR group), 6 patients had a 1 + 1/2 ventricular repair (1 + 1/2V group) and 5 patients had a Fontan completion (Fontan group). After modified BTS with pulmonary valvotomy, tricuspid valve z-score did not increase in any of the group (BVR: pre -2.79 vs post -2.24, 1 + 1/2V: pre -5.25 vs post -6.69, Fontan: pre -6.82 vs post -7.94). Normalized RV end-diastolic volume increased only in BVR group after modified BTS with pulmonary valvotomy (BVR: pre 32% vs post 64%, 1 + 1/2V: pre 43% vs post 42%, Fontan: pre 29% vs post 32%). Major RV-coronary artery fistula was a strong factor with proceeding single-ventricle palliation [BVR: 4/30 (13%) patients, 1 + 1/2V: 1/6 (17%) and Fontan: 4/5 (80%)]. CONCLUSIONS: Tricuspid valve growth was not obtained by modified BTS with pulmonary valvotomy; therefore, tricuspid valve size at birth appeared to be a predictor for achieving BVR. Proportionate RV growth was seen only in patients who achieved BVR. However, RV growth was not seen in patients having 1 + 1/2 ventricular repair. Major RV-coronary artery fistula was a strong predictor for proceeding single-ventricle palliation.

DOI： 10.1093/ejcts/ezw124

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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OBJECT: We compared the clinical effectiveness and biocompatibility of poly-2-methoxyethyl acrylate (PMEA)-coated and heparin-coated cardiopulmonary bypass (CPB) circuits in a prospective pediatric trial. METHODS: Infants randomly received heparin-coated (n=7) or PMEA-coated (n=7) circuits in elective pediatric cardiac surgery with CPB for ventricular septum defects. Clinical and hematologic variables, respiratory indices and hemodynamic changes were analyzed perioperatively. RESULTS: Demographic and clinical variables were similar in both groups. Leukocyte counts were significantly lower 5 minutes after CPB in the PMEA group than the heparin group. Hemodynamic data showed that PMEA caused hypotension within 5 minutes of CPB. The respiratory index was significantly higher immediately after CPB and 1 hour after transfer to the intensive care unit (ICU) in the PMEA group, as were levels of C-reactive protein 24 hours after transfer to the ICU. CONCLUSION: Our study shows that PMEA-coated circuits, unlike heparin-coated circuits, cause transient leukopenia during pediatric CPB and, perhaps, systemic inflammatory respiratory syndrome after pediatric CPB.

DOI： 10.1177/0267659115598217

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Language：Japanese   Publisher：岡山医学会  

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DOI： 10.1177/0218492314542288

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The objective of this study was to compare the effects of pulsatile and nonpulsatile extracorporeal membrane oxygenation (ECMO) on hemodynamic energy and systemic microcirculation in an acute cardiac failure model in piglets. Fourteen piglets with a mean body weight of 6.08 ± 0.86 kg were divided into pulsatile (N = 7) and nonpulsatile (N = 7) ECMO groups. The experimental ECMO circuit consisted of a centrifugal pump, a membrane oxygenator, and a pneumatic pulsatile flow generator system developed in-house. Nonpulsatile ECMO was initiated at a flow rate of 140 mL/kg/min for the first 30 min with normal heart beating, with rectal temperature maintained at 36°C. Ventricular fibrillation was then induced with a 3.5-V alternating current to generate a cardiac dysfunction model. Using this model, we collected the data on pulsatile and nonpulsatile groups. The piglets were weaned off ECMO at the end of the experiment (180 min after ECMO was initiated). The animals did not receive blood transfusions, inotropic drugs, or vasoactive drugs. Blood samples were collected to measure hemoglobin, methemoglobin, blood gases, electrolytes, and lactic acid levels. Hemodynamic energy was calculated using the Shepard's energy equivalent pressure. Near-infrared spectroscopy was used to monitor brain and kidney perfusion. The pulsatile ECMO group had a higher atrial pressure (systolic and mean), and significantly higher regional saturation at the brain level, than the nonpulsatile group (for both, P < 0.05). Additionally, the pulsatile ECMO group had higher methemoglobin levels within the normal range than the nonpulsatile group. Our study demonstrated that pulsatile ECMO produces significantly higher hemodynamic energy and improves systemic microcirculation, compared with nonpulsatile ECMO in acute cardiac failure.

DOI： 10.1111/aor.12588

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OBJECTIVES: Our aim was to assess midterm safety and clinical outcomes of intracoronary infusion of cardiosphere-derived cells (CDCs) after staged palliation in patients with hypoplastic left heart syndrome (HLHS). METHODS: In this prospective, controlled study, 14 consecutive patients with HLHS who were undergoing 2- or 3-stage surgical palliations were assigned to receive intracoronary CDC infusion 1 month after cardiac surgery (n = 7), followed by 7 patients allocated to a control group with standard care alone. The primary end point was to assess procedural feasibility and safety; the secondary end point was to evaluate cardiac function and heart failure status through 36-month follow-up. RESULTS: No complications, including tumor formation, were reported within 36 months after CDC infusion. Echocardiography showed significantly greater improvement in right ventricular ejection fraction (RVEF) in infants receiving CDCs than in controls at 36 months (+8.0% ± 4.7% vs +2.2% ± 4.3%; P = .03). These cardiac function improvements resulted in reduced brain natriuretic peptide levels (P = .04), lower incidence of unplanned catheter interventions (P = .04), and higher weight-for-age z score (P = .02) at 36 months relative to controls. As independent predictors of treatment responsiveness, absolute changes in RVEF at 36 months were negatively correlated with age, weight-for-age z score, and RVEF at CDC infusion. CONCLUSIONS: Intracoronary CDC infusion after staged procedure in patients with HLHS is safe and improves RVEF, which persists during 36-month follow-up. This therapeutic strategy may enhance somatic growth and reduce incidence of heart failure.

DOI： 10.1016/j.jtcvs.2015.06.076

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BACKGROUND: The surgical outcomes of hypoplastic left heart syndrome with intact atrial septum remains very poor in spite of the introduction of prenatal diagnosis before the Norwood operation. The hybrid operation consisting of bilateral pulmonary artery banding and balloon atrioseptectomy is one of the treatment strategies to potentially improve this patient population, however, the long-term outcomes are unknown. METHODS: Six consecutive patients with hypoplastic left heart syndrome with intact atrial septum, who underwent the hybrid operation between October 2006 and July 2014, were retrospectively reviewed. Hypoplastic left heart syndrome with highly restrictive atrial communication was excluded. RESULTS: Three patients died after the hybrid operation, due to sepsis, cerebral bleeding, and heart failure. Three patients underwent the modified Norwood operation. One of these died due to severe hypoxia while awaiting the bidirectional Glenn operation. The others underwent a bidirectional Glenn operation and subsequent Fontan completion but died due to lung dysfunction and sudden hemoptysis. CONCLUSIONS: The hybrid operation for hypoplastic left heart syndrome with intact atrial septum may have improved the neonatal outcome and Fontan completion rate in this extremely high-risk group of patients, but the long-term outcome remains dismal. Considering the late mortality related to pulmonary complications, aggressive fetal intervention to create a nonrestrictive atrial septal communication to promote normal development of the pulmonary vessels may be the last resort to improve the long-term outcome.

DOI： 10.1177/0218492315606581

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Myxoma is the most common primary cardiac tumor in adults; however, it is extremely rare in infants. Acute occlusion of the abdominal aorta by a cardiac myxoma is also rare. We report the case of an infant with acute occlusion of the suprarenal abdominal aorta by a left ventricular myxoma. The patient underwent successful catheter embolectomy of the abdominal aorta and surgical resection of the cardiac myxoma. This is a very rare case report of the combination of infantile left ventricular myxoma and acute occlusion of the abdominal aorta.

DOI： 10.1016/j.athoracsur.2014.08.066

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

Fontan循環不全の心停止患者に対してV-A extracorporeal mambrane oxygenation(ECMO)が奏功した1症例を経験したので報告する。患者は40歳の男性で身長161cm、体重55.6kg、体表面積1.48m2であった。2013年4月からFontan循環不全による蛋白漏出性胃腸症および腹水貯留を認め、Biderectional Glenn(BDG)とmodified B-T shunt(m-BTS)手術へ移行する予定で同年5月に当院へ入院した。入院中に痙攣発作から心停止となりECMOを導入した。ECMOは右総頸動脈送血、左大腿静脈脱血にて行い灌流量は2.4L/min/m2で管理した。ECMO導入後は循環動態が安定し中心静脈圧は20mmHgから10mmHgへ減少した。ECMO導入後に神経障害、多臓器不全を認めなかった。Fontan循環不全の心停止患者に対してECMOを導入することによって静脈圧を下げることができ、安定した全身循環を保ちつつBDGとm-BTS手術へ移行することが可能であった。(著者抄録)

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Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

頻脈誘発性心筋症(tachycardia induced cardiomyopathy:TIC)による心原性ショックに対してExtracorporeal membrane oxygenation(ECMO)を導入し奏功した1症例を経験したので報告する。患者は2歳10ヵ月の女児で身長84.5cm、体重10kgであった。生後1ヵ月に多発性筋性部心室中隔欠損症、多源性心房頻拍と診断され、生後2ヵ月で肺動脈絞扼術を施行した。2012年10月に心拍数190回/分、収縮期血圧50mmHg、拡張期血圧35mmHg、平均血圧40mmHg、心臓超音波検査で左室駆出率(left ventricular ejection fraction:LVEF)が13%、無尿で心原性ショック状態であり抗不整脈薬を投与しても改善が認められないためECMOを導入した。ECMO導入後も抗不整脈薬の投与を継続した。ECMO導入後に心拍数、左室拡張末期径と血清脳性ナトリウム利尿ペプチドの減少、更にLVEFの改善を認めたことからTICと診断した。ECMOにより循環動態を保ちながら、抗不整脈薬の投与を継続したことで心拍数の正常化を得ることができ、心機能の改善を認めたと考える。(著者抄録)

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is now a candidate therapy for children with acute respiratory failure. CASE PRESENTATION: We report our experience of using central ECMO therapy for acute respiratory distress syndrome followed by seizure in a 15-month-old girl with a severe epileptic disorder. Her respiratory distress was refractory to standard medical treatment and mechanical ventilatory support. Her condition was complicated by development of a pneumothorax. The patient was successfully weaned off ECMO and discharged without deterioration of her neurological status. CONCLUSION: The successful outcome in this case resulted from the central ECMO, which enabled "lung rest" and adequate cerebral blood flow. In skilled ECMO facilities, early implementation of ECMO would give some advantages to patients such as the one presented here. Given the invasiveness and the ease of the procedure, introduction of dual-lumen catheters adequately sized for pediatric patients in Japan is required.

DOI： 10.1186/s12887-015-0348-1

PubMed

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Language：Japanese   Publisher：(一社)日本外科学会  

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RATIONALE: Hypoplastic left heart syndrome (HLHS) remains a lethal congenital cardiac defect. Recent studies have suggested that intracoronary administration of autologous cardiosphere-derived cells (CDCs) may improve ventricular function. OBJECTIVE: The aim of this study was to test whether intracoronary delivery of CDCs is feasible and safe in patients with hypoplastic left heart syndrome. METHODS AND RESULTS: Between January 5, 2011, and January 16, 2012, 14 patients (1.8±1.5 years) were prospectively assigned to receive intracoronary infusion of autologous CDCs 33.4±8.1 days after staged procedures (n=7), followed by 7 controls with standard palliation alone. The primary end point was to assess the safety, and the secondary end point included the preliminary efficacy to verify the right ventricular ejection fraction improvements between baseline and 3 months. Manufacturing and intracoronary delivery of CDCs were feasible, and no serious adverse events were reported within the 18-month follow-up. Patients treated with CDCs showed right ventricular ejection fraction improvement from baseline to 3-month follow-up (46.9%±4.6% to 52.1%±2.4%; P=0.008). Compared with controls at 18 months, cardiac MRI analysis of CDC-treated patients showed a higher right ventricular ejection fraction (31.5%±6.8% versus 40.4%±7.6%; P=0.049), improved somatic growth (P=0.0005), reduced heart failure status (P=0.003), and lower incidence of coil occlusion for collaterals (P=0.007). CONCLUSIONS: Intracoronary infusion of autologous CDCs seems to be feasible and safe in children with hypoplastic left heart syndrome after staged surgery. Large phase 2 trials are warranted to examine the potential effects of cardiac function improvements and the long-term benefits of clinical outcomes. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01273857.

DOI： 10.1161/CIRCRESAHA.116.304671

PubMed

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BACKGROUND: Congenital heart diseases often involve chronic pressure overload of the right ventricle (RV) which is a major cause of RV dysfunction. Pulmonary artery (PA) banding has been used to produce animal models of RV dysfunction. We have devised a new and easier method of constricting the PA and compared it directly with the partial ligation method. METHODS: Eight-week-old male Sprague-Dawley rats (240-260 g) were divided into three groups: sham operation, partial pulmonary artery ligation (PAL) procedure, and pulmonary artery half-closed clip (PAC) procedure. RV function and remodeling were determined by echocardiography and histomorphometry. RESULTS: Surgical mortality was significantly lower in the PAC group while echocardiography revealed significantly more signs of RV dysfunction. At the 8th week after surgery RV fibrosis rate was significantly higher in the PAC group. CONCLUSIONS: This procedure of pulmonary artery banding in rats is easier and more efficient than partial ligation.

DOI： 10.1155/2015/753210

PubMed

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Language：Japanese   Publishing type：Research paper (scientific journal)  

The Norwood procedure with right ventricle-pulmonary artery (RV-PA) conduit is thought to improve post-operative hemodynamics in hypoplastic left heart syndrome( HLHS). Prematurity( GA<37 weeks), body weight less than 2.5 kg and tricuspid regurgitation( TR)2+ or more were associated with mortality. For requiring better outcome, paralyzed ventilation, inherent N2 are indicated, to avoid preoperative high pulmonary blood flow followed by progressive TR. Rapid staged bilateral pulmonary artery banding( PAB) might improve the outcome for this high mortality group. As operative improvement, the distal neoaortic reconstruction was completed without any patch materials in most case. For achievement this, it is important that aortic arch and ascending aorta just opposite the site of the innominate artery were sutured inferiorly to extend the width of the aortic arch. Also we have developed a modified strategy to control RV-PA shunt flow, which is partial clipping on the ePTFE graft. Because significant TR associated with mortality, TR should be surgically treated at any sage of reconstruction. At bidirectional Glenn operation, 70% of patient were left the RV-PA conduit as additional flow. Hospital mortality after Fontan completion for HLHS is excellent with short averaging more than 95%. Extracardiac conduit with fenestration is indicated in all HLHS patients.

PubMed

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OBJECTIVES: In cases of severe Ebstein's anomaly, it is essential to determine whether biventricular repair (BVR) or single-ventricle palliation is feasible. Since 1999, in our institution, we have used the novel technique comprising tricuspid valve (TV) closure and right ventricular and right atrial (RV/RA) exclusion to reduce the deleterious effects of an enlarged RV in patients with severe Ebstein's anomaly. However, in cases with good RV function, primary BVR is performed. In the present study, we describe our surgical strategy in the treatment of severely symptomatic neonates with Ebstein's anomaly. METHODS: From June 1999 to October 2011, 12 neonates with a severely symptomatic Ebstein's anomaly underwent surgical repair. The mean age at the first operation was 29 ± 25 (range, 5-92) days; and the mean body weight was 2.8 ± 0.5 (range, 2.0-4.1) kg. The associated anomalies included pulmonary atresia with an intact ventricular septum in 7, critical pulmonary stenosis in 1, ventricular septal defect in 3 and coarctation of the aorta in 1 patient. The mean cardio-thoracic ratio (CTR) was 80 ± 14% (range, 57-98%). Preoperatively, 9 patients had grade IV tricuspid regurgitation (TR), as detected by echocardiography, and 6 required ventilator support. RESULTS: Five patients underwent primary BVR. Seven patients underwent staged palliation using a modified Blalock-Taussig shunt (BT shunt) with/without RV/RA exclusion. There was 1 case each of hospital death and late death. The median follow-up duration in the present study was 6.5 years. Among the 8 patients who underwent TV repair, postoperative TR was trivial or mild in 6 patients, moderate in 1 and absent in 1. After surgery, the mean CTR and serum B-type natriuretic peptide levels decreased to 59 ± 14% (range, 45-70%) and 46 ± 28 (range, 12-83) pg/dl, respectively. CONCLUSIONS: Critically ill neonates with Ebstein's anomaly can be successfully treated using RV/RA exclusion combined with a modified BT shunt in cases where RV function is poor. However, in cases of good RV function, we recommend the use of primary BVR.

DOI： 10.1093/ejcts/ezt465

PubMed

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The genetic basis of hypoplastic left heart syndrome (HLHS) remains unknown, and the lack of animal models to reconstitute the cardiac maldevelopment has hampered the study of this disease. This study investigated the altered control of transcriptional and epigenetic programs that may affect the development of HLHS by using disease-specific induced pluripotent stem (iPS) cells. Cardiac progenitor cells (CPCs) were isolated from patients with congenital heart diseases to generate patient-specific iPS cells. Comparative gene expression analysis of HLHS- and biventricle (BV) heart-derived iPS cells was performed to dissect the complex genetic circuits that may promote the disease phenotype. Both HLHS- and BV heart-derived CPCs were reprogrammed to generate disease-specific iPS cells, which showed characteristic human embryonic stem cell signatures, expressed pluripotency markers, and could give rise to cardiomyocytes. However, HLHS-iPS cells exhibited lower cardiomyogenic differentiation potential than BV-iPS cells. Quantitative gene expression analysis demonstrated that HLHS-derived iPS cells showed transcriptional repression of NKX2-5, reduced levels of TBX2 and NOTCH/HEY signaling, and inhibited HAND1/2 transcripts compared with control cells. Although both HLHS-derived CPCs and iPS cells showed reduced SRE and TNNT2 transcriptional activation compared with BV-derived cells, co-transfection of NKX2-5, HAND1, and NOTCH1 into HLHS-derived cells resulted in synergistic restoration of these promoters activation. Notably, gain- and loss-of-function studies revealed that NKX2-5 had a predominant impact on NPPA transcriptional activation. Moreover, differentiated HLHS-derived iPS cells showed reduced H3K4 dimethylation as well as histone H3 acetylation but increased H3K27 trimethylation to inhibit transcriptional activation on the NKX2-5 promoter. These findings suggest that patient-specific iPS cells may provide molecular insights into complex transcriptional and epigenetic mechanisms, at least in part, through combinatorial expression of NKX2-5, HAND1, and NOTCH1 that coordinately contribute to cardiac malformations in HLHS.

DOI： 10.1371/journal.pone.0102796

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We report the effect of pulmonary root translocation on the left ventricular outflow tract. A double switch operation with pulmonary root translocation was performed in a 6-year-old boy whose diagnosis was dextrocardia, congenitally corrected transposition of the great arteries, ventricular septal defect, and pulmonary stenosis. Postoperative magnetic resonance imaging showed more natural left ventricular outflow than preoperatively (19 mm vs 22 mm in length between the top of the interventricular septum and the aortic valve). This technique does not require coronary transfer and enables preservation of the aortic root structure. The long-term results, including left ventricular outflow tract morphology, should be evaluated.

DOI： 10.1016/j.athoracsur.2013.02.050

PubMed

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Language：Japanese   Publisher：Japanese Society of Pediatric Cardiology and Cardiac Surgery  

Congenital ventricular aneurysm is a very rare heart anomaly，especially when it occurs in the right ventricle. It is associated with a poor prognosis and a risk of sudden death. We report a case of congenital right ventricular aneurysm that was successfully repaired by surgery. In this case，echocardiography at birth revealed an aneurysmal change at the right ventricular apex. Rapid enlargement of this aneurysm was detected during careful follow-up，and surgical repair was performed at six months of age. The postoperative course was uneventful，and the patient was free from symptoms at 10 months after surgery.

DOI： 10.9794/jspccs.29.194

CiNii Article

CiNii Books

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Other Link： http://search.jamas.or.jp/link/ui/2013371520

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: We sought to evaluate the efficacy of balloon angioplasty (BA) for severely desaturated patients due to a stenotic right ventricle (RV) to pulmonary artery (PA) shunt following modified Norwood procedure. METHODS: Of 87 patients who underwent a Norwood procedure with the RV-PA shunt between February 1998 through March 2010, 22 (25%) patients underwent BA. The efficacy of BA was assessed by angiographic measurement of the changes in the internal diameters of the stenotic portions of the shunt, changes in arterial saturation and clinical outcomes. RESULTS: BA was performed for stenotic RV-PA shunts following stage I palliation (n = 17, 77%), or those placed as an additional blood source (n = 5, 23%, 3 patients awaiting biventricular repair, 2 patients following stage II palliation). The location of the BA was at the distal anastomosis in 12 (54.5%), proximal anastomosis in 21 (95.4%) and in the mid-portion of the shunt in 11 (50%) cases. The diameters of these three shunt portions were measured from the anterior-posterior and lateral angiographic images, increasing significantly after BA (p < 0.0001) in all. Arterial saturation significantly improved after BA in all cases (66.5 ± 4.3% to 79.4 ± 3.4%, p < 0.0001). Freedom from reintervention was 100%. All patients underwent subsequent elective planned surgery at an appropriate age with no mortality. CONCLUSIONS: A BA-alone strategy for a stenotic RV-PA shunt was effective for all three shunt portions, minimizing shunt-related premature surgical intervention.

DOI： 10.1002/ccd.24576

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Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

両大血管右室起始症に対する外科治療法の違いが体外循環法に及ぼす影響について考察した。当院で初回介入手術を行った両大血管右室起始と診断のついた39例を対象として、二心室修復群(n=16)と単心室修復群(n=23)を比較した。二心室修復群で体外循環時間、大動脈遮断時間、挿管時間が有意に長かった。二心室修復群は術後のヘマトクリット値、局所酸素飽和度、拡張期圧、総蛋白量、MUF中の中心静脈圧変化率が有意に高かった。単心室修復群の術前後で中心静脈圧が有意に高かった。無輸血体外循環達成率は二心室修復群44%、単心室修復群78%であった。単心室修復群は無輸血体外循環達成率が高かったが、容量負荷による血行動態の管理を必要としたためMUFの除水効率および血液濃縮効果は低く、局所酸素飽和度も低い傾向にあった。両大血管右室起始に対する単心室修復術は二心室修復術に比べて容量依存型循環エネルギーによる血行動態管理、水分バランス制御による体外循環管理を必要とした。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2012&ichushi_jid=J01893&link_issn=&doc_id=20121218330003&doc_link_id=%2Fcz4jsect%2F2012%2F003904%2F003%2F0428-0431%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fcz4jsect%2F2012%2F003904%2F003%2F0428-0431%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：English   Publishing type：Research paper (scientific journal)   Publisher：CHURCHILL LIVINGSTONE INC MEDICAL PUBLISHERS  

DOI： 10.1016/j.cardfail.2012.08.116

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Language：Japanese   Publisher：(一社)香川県医師会  

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Extracorporeal membrane oxygenation (ECMO) has emerged as an effective mechanical support following cardiac surgery with respiratory and cardiac failure. However, there are no clear indications for ECMO use after pediatric cardiac surgery. We retrospectively reviewed medical records of 76 pediatric patients [mean age, 10.8 months (0-86); mean weight, 5.16 kg (1.16-16.5)] with congenital heart disease who received ECMO following cardiac surgery between January 1997 and October 2010. Forty-five patients were treated with an aggressive ECMO approach (aggressive ECMO group, April 2005-October 2010) and 31 with a delayed ECMO approach (delayed ECMO group, January 1997-March 2005). Demographics, diagnosis, operative variables, ECMO indication, and duration of survivors and non-survivors were compared. Thirty-four patients (75.5%) were successfully weaned from ECMO in the aggressive ECMO group and 26 (57.7%) were discharged. Conversely, eight patients (25.8%) were successfully weaned from ECMO in the delayed ECMO group and two (6.5%) were discharged. Forty-five patients with shunted single ventricle physiology (aggressive: 29 patients, delayed: 16 patients) received ECMO, but only 15 (33.3%) survived and were discharged. The survival rate of the aggressive ECMO group was significantly better when compared with the delayed ECMO group (p<0.01). Also, ECMO duration was significantly shorter among the aggressive ECMO group survivors (96.5 ± 62.9 h, p<0.01). Thus, the aggressive ECMO approach is a superior strategy compared to the delayed ECMO approach in pediatric cardiac patients. The aggressive ECMO approach improved our outcomes of neonatal and pediatric ECMO.

DOI： 10.1177/0267659111434857

PubMed

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Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

心室中隔欠損症に対する肺高血圧症の合併の有無が体外循環法にどのような影響を与えるのかについて後方視的研究を行った。乳児期に心室中隔欠損症に対する手術を施行した28症例を対象とし、肺高血圧症合併群(PH群)14例と非合併群(N群)14例に分類し比較検討した。PH群では挿管時間、集中治療室の滞在時間が有意に長かった。PH群では体外循環中の限外濾過量が多かった。PH群では体外循環後の収縮期/拡張期血圧が低かった。PH群では術前に肺動脈弁輪径および左室拡張末期径が拡大し、術後には右室拡張末期径が拡大していた。PH群では術前に肺動脈弁が拡大し肺動脈血流の増加に伴う容量負荷が認められ、術後には容量負荷による右心不全傾向と呼吸不全の傾向が認められた。これらのことから、肺動脈圧を合併した心室中隔欠損症に対する乳児体外循環の際には、容量負荷に対応した体外循環法が求められ、体外循環後の肺高血圧危機の予防と右心不全対策、また術後の呼吸機能低下に対する対策を考慮した体外循環法の確立が望まれる。(著者抄録)

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Language：Japanese   Publisher：(株)学研メディカル秀潤社  

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Heterotaxy syndrome is a mortality risk factor for patients with complex heart disease. These patients often have common atrioventricular valve (CAVV) morphology and significant atrioventricular valve regurgitation - yet another mortality risk factor in congenital heart disease. Thus, patients with both heterotaxy syndrome and atrioventricular valve regurgitation are at highest risk of mortality. A large number of patients with heterotaxy syndrome have CAVV and a functional single ventricle. These patients are more difficult to operate than patients with biventricular morphology because in the former, the CAVV is often less adapted to systemic afterload than the mitral valve. Herein, we present the results of atrioventricular valve repair surgeries performed at the Okayama University Hospital on patients with heterotaxy syndrome, atrial isomerism, and a functional single ventricle. In addition, we review the current literature in this typically challenging patient population. In-depth understanding of reasonable management strategies and resolution of technical issues will help guide surgeons during this procedure.

DOI： 10.1053/j.pcsu.2012.01.014

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DOI： 10.1053/j.optechstcvs.2012.07.001

Scopus

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DOI： 10.1016/j.jtcvs.2011.05.019

PubMed

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OBJECTIVE: The Damus-Kaye-Stansel operation sometimes results in deteriorating semilunar valve insufficiency. We verified the semilunar valve function after the Damus-Kaye-Stansel operation and compared the end-to-side Damus-Kaye-Stansel with the double-barrel Damus-Kaye-Stansel. METHODS: Forty-seven patients who underwent the Damus-Kaye-Stansel operation between June 1993 and August 2008 were retrospectively reviewed. Any patient who underwent a Norwood-type operation was excluded. The median age at operation was 19 months (range, 0-276 months). Forty-five patients were Fontan candidates. Thirty-nine patients underwent pulmonary artery banding before the Damus-Kaye-Stansel operation. Twenty-two patients had undergone an arch repair previously. The semilunar valve function was evaluated by echocardiography. RESULTS: Thirteen patients underwent the end-to-side Damus-Kaye-Stansel operation, and 34 patients underwent the double-barrel Damus-Kaye-Stansel operation. The mean follow-up period was 71 ± 50 months (range, 1-188 months). Although there were 4 deaths, no death was related to the Damus-Kaye-Stansel procedure. Two of the patients with early death could not undergo a postoperative evaluation of the semilunar valves. The semilunar valve regurgitation mildly deteriorated in 7 patients (pulmonary regurgitation in 5 patients and aortic regurgitation in 2 patients). Pulmonary regurgitation deteriorated from none to mild in 1 patient, none to trivial in 2 patients, and trivial to mild in 2 patients. Both deteriorations in aortic regurgitation ranged from none to trivial. Semilunar valve regurgitation did not affect patients' circulatory condition. The end-to-side Damus-Kaye-Stansel operation more frequently caused a deterioration in pulmonary regurgitation than the double-barrel Damus-Kaye-Stansel operation (4/11 vs 1/34, P = .001). No surgical intervention for a systemic ventricular outflow obstruction was observed in the follow-up period. CONCLUSIONS: The double-barrel Damus-Kaye-Stansel operation was found to be superior to the end-to-side Damus-Kaye-Stansel operation for the prevention of postoperative pulmonary regurgitation.

DOI： 10.1016/j.jtcvs.2010.06.007

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Extracorporeal membrane oxygenation (ECMO) is an important circulatory assist for children with refractory cardiopulmonary dysfunction, but its role and indications after a stage 1 Norwood procedure are controversial. We assessed outcomes and risk factors in patients who underwent a Norwood palliation and ECMO at our institution. We retrospectively reviewed all patients who underwent a Norwood procedure and were supported with ECMO between January 1998 and January 2010. Of the 91 children who underwent a Norwood procedure during the study period, there were 15 postoperative runs of ECMO in 12 patients. The diagnoses of the patients included five with hypoplastic left heart syndrome, five with a hypoplastic left heart syndrome variant, and two with critical aortic stenosis. A total of four patients underwent bilateral pulmonary artery banding, and two patients underwent aortic valvuloplasty before the stage 1 Norwood procedure. The mean age of the patients was 28±30 days, and mean body weight was 2.6±0.5kg at the induction of ECMO. The indications for ECMO were low cardiac output in six children, circulatory collapse needing cardiopulmonary resuscitation in six children, and hypoxemia in three children. Five of the 12 patients were successfully weaned from ECMO. The significant risk factors for the inability to be weaned from ECMO were a history of circulatory collapse requiring cardiopulmonary resuscitation, and the induction of ECMO in the intensive care unit. Induction of ECMO may be considered earlier when hemodynamics are unstable in impaired patients following a stage 1 Norwood procedure to avoid circulatory collapse.

DOI： 10.1111/j.1525-1594.2010.01141.x

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Language：Japanese   Publisher：膜型人工肺研究会  

小児心臓手術後にECMOを積極的に導入した群(37例)と、ECMOの導入を出来るだけ遅らせた群(31例)とで術後成績を比較検討した。その結果、積極的導入群のほうが明らかに成績良好であり、ECMO離脱率も積極的導入群が有意に高かった。

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Language：Japanese   Publisher：膜型人工肺研究会  

新型インフルエンザ罹患によって急性呼吸窮迫症候群をきたした患者に対して、ECMOを導入し呼吸状態と循環動態の改善を図った結果、全身状態が劇的に回復し救命することができた症例を報告した。患者は11歳女児で、ECMOの早期導入によりLung Restが得られたことで、人工呼吸療法に起因する肺障害を回避できたと考えられた。

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There is still no consensus on the optimal management to treat pediatric mediastinitis. We assessed the efficacy of continuous irrigation and vacuum-assisted closure (VAC) for mediastinitis in children. This study retrospectively reviewed 20 patients aged <5 years with mediastinitis from December 2002 to December 2009. The median age at the onset was 12 months (0.6-60 months), and the median body weight was 6.9 kg (3.1-15.3 kg). Continuous irrigation was applied for extensive mediastinitis or unstable hemodynamic cases and VAC for localized or ineffective cases after continuous irrigation. A 2-4-week course of intravenous antibiotics was administered after sternal closure. Continuous irrigation was initially applied in 19 patients and VAC in one patient. VAC was employed in six patients because of recurrent or prolonged mediastinitis after continuous irrigation. All patients underwent direct sternal closure without any flap. The median duration of the hospital stay was 49.5 days (15-158 days). Although two patients died of low cardiac output, 18 children survived and had no recurrence after the discharge during a median follow-up of 14 months (1-81 months). The combination of continuous irrigation and VAC is, therefore, considered to be a safe and effective option to minimize the morbidity and mortality in pediatric mediastinitis.

DOI： 10.1510/icvts.2010.235903

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The anatomy of a persistent left superior vena cava (SVC) to the left atrium (LA) without the innominate vein can make it challenging to complete intracardiac repair. We reviewed our five cases of the direct end-to-side anastomosis of SVCs to facilitate anatomical repair of SVC-right atrial connection for biventricular repair. Diagnoses were two partial atrioventricular septal defect with left isomerism, one complete atrioventricular septal defect (CAVSD) with left isomerism, one CAVSD without isomerism and one atrioventricular discordance and double outlet right ventricle with right isomerism. Mean age at the operation was 20+/-23 months (4-58 months) and body weight was 7.8+/-3.4 kg (4.8-12.7 kg). After completion of intracardiac repair, the SVC to LA was divided and end-to-side anastomosed to the SVC to the right atrium during cardiopulmonary bypass. No early or late death occurred during follow-up of 14.4+/-6.9 months (7-23 months). None of the patients developed an obstruction at the anastomosis site of the SVCs. The direct end-to-side anastomosis of SVCs achieved an excellent anatomical SVC-right atrium connection in complex congenital heart diseases.

DOI： 10.1510/icvts.2009.230581

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Aortic root dilatation is rare in children, and is often secondary to Marfan syndrome (MFS). We experienced a case of a three-year-old boy (92 cm, 12 kg) with MFS presenting with progressive dilatation of aortic root. We electively performed a valve-sparing aortic root replacement using a 24-mm Gelweave Valsalva graft. Although the patient required a mitral valve repair due to infective endocarditis postoperatively, the recovery from the second surgery was uneventful. This case is one of the youngest children of valve-sparing aortic root replacement in the literature.

DOI： 10.1510/icvts.2010.234138

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OBJECTIVE: A lateral tunnel-total cavopulmonary connection (LT-TCPC) using a right atrial (RA) free wall is the first choice of treatment for patients with a small body weight in this institute. Whether the growth of the LT is appropriate or not according to the growth of the body remains controversial. In addition, the optimal initial diameter of an LT is unknown. The purpose of this study was to verify the growth of the LT. METHODS: Ninety-one patients of a total of 267 TCPC cases underwent an LT-TCPC at less than 5 years of age in this institute between March 1991 and June 2008. The data on 47 of the 91 patients, which were available to investigate the LT growth, were retrospectively reviewed. The mean age at LT-TCPC was 37+/-11 months (16-57 months). The mean body weight at TCPC was 12.4+/-2.4 kg (7.6-20.0 kg). The initial LT diameter was determined with Hegar's sizer of the estimated half-pulmonary arterial (PA) diameter, which is a diameter that results in half of the dimension of the normal pulmonary valve. The measured maximum LT diameter (mm) divided by the estimated half-PA diameter (mm) was considered as the LT index. The size of the LT was evaluated using either echocardiography or angiography. RESULTS: The mean follow-up period was 7.4+/-3.5 years (1.6-13.5 years). The LT index was initially 2.0+/-0.7 (1.3-4.5), 2.0+/-0.4 (1.3-3.2) at 1 year after operation, 2.1+/-0.5 (1.5-3.2) at 5 years after operation, 1.9+/-0.4 (1.5-2.8) at 10 years after operation and 2.1+/-0.5 (1.6-2.5) at 13 years after operation, respectively. CONCLUSIONS: LT growth suitable for the body growth can be expected. Although there was some variation in the initial LT diameter, the LT index tended to converge at 2.0 with growth.

DOI： 10.1016/j.ejcts.2010.01.014

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The purpose of this study was to establish a useful cut-off level for performing an original Rastelli-type operation in patients with transposition of the great arteries (TGA)/ventricular septal defect (VSD) or double outlet right ventricle (DORV). A total of 43 patients with TGA/VSD or DORV who underwent an original Rastelli-type operation in this institute between March 1993 and January 2009 were reviewed retrospectively. These patients were divided into two groups using the length between the top of the interventricular septum and the aortic valve (IVS-AV length); Group A; IVS-AV length <80% of normal left ventricular end-diastolic diameter (LVDd). Group B; IVS-AV length > or =80% of normal LVDd. Group A had a significantly better survival than Group B (100% vs. 56%, P=0.001). The cardiac event-free survival were 89.1% at 7.2 years in Group A and 26.3% at 8.4 years in Group B (P<0.0001). The Group B had a higher incidence of left ventricular outflow tract obstruction (LVOTO; 3% vs. 33%, P=0.02). The IVS-AV length was found to be a significant risk factor for mortality and LVOTO. The IVS-AV length should, therefore, be taken into consideration when selecting the optimal surgical procedures for these patients.

DOI： 10.1510/icvts.2009.223982

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DOI： 10.1016/j.jtcvs.2009.02.056

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Language：Japanese   Publisher：Japan Heart Foundation  

DOI： 10.11281/shinzo.42.1450

CiNii Article

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Other Link： http://search.jamas.or.jp/link/ui/2011049254

Language：Japanese   Publisher：(一社)日本体外循環技術医学会編集委員会  

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Careful rewarming of perfusion blood following cardiopulmonary bypass surgery is critical to a successful outcome, but the optimal rewarming strategy is not clear. The purpose of this study was to derive a formula for a rewarming index (defined as [rewarming time x perfusion flow]/[body weight x body surface area]) that would enable the calculation of the ideal rewarming conditions for pediatric cardiopulmonary perfusion. We retrospectively investigated 220 pediatric cardiopulmonary bypass operations conducted from July 2005 to June 2008 in Okayama University Hospital, Japan. We determined the formula as Phi = (T x Q)/(R x S) = |0.9127P - 0.0152|, where Phi = rewarming index, T = rewarming time (min), Q = perfusion volume (L), R = body weight (kg), S = body surface area (m(2)), and P = temperature gap (right angle). The formula will help those who perform pediatric cardiopulmonary bypass surgery to establish ideal perfusion flow conditions and to control physiological temperature during rewarming.

DOI： 10.1111/j.1525-1594.2009.00945.x

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OBJECTIVE: Tricuspid valve size is the major determinant of outcomes for patients with pulmonary atresia with intact ventricular septum. Lack of right ventricle-pulmonary artery continuity is associated with poor tricuspid valve growth (decrement in Z-value). However, most reports did not show evidence for disproportionate growth of the tricuspid valve after establishment of right ventricle-pulmonary artery continuity. METHODS: We studied 40 patients with pulmonary atresia with intact ventricular septum who underwent initial right ventricular decompression for planned staged repair. The initial Z-value of the tricuspid valve diameter (Zt1) was obtained from the echocardiography-derived normal value. The late Z-value (Zt2) was measured before definitive repair or the last available Z-value, if definitive repair was not yet reached. The factors associated with the changes of Z-values (Zt2 - Zt1) were analyzed. RESULTS: The mean initial tricuspid Z-value (Zt1) was -6.2 +/- 3.5. After treatment (Zt2), the mean Z-value was -6.0 +/- 3.4 (n = 34). Overall, the tricuspid Z-values did not change. Individually, the change in Z-value (Zt2 - Zt1) was larger than +2 in 11 (32%) patients and smaller than -2 in 6 (18%) patients. Increases in Z-value (Zt2 - Zt1) were significantly associated with right ventricular pressure/left ventricular pressure ratio measured after initial palliation (r = -0.54; P = .001) and the initial tricuspid valve Z-value (Zt1) (r = -0.40; P = .02). CONCLUSIONS: Disproportional growth of the tricuspid valve can occur, especially in patients with small tricuspid valves and lower right ventricular pressures after decompression. The findings support the possibility of neonates with small tricuspid valves undergoing biventricular repair after right ventricular decompression surgery.

DOI： 10.1016/j.jtcvs.2009.05.015

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The high-flow management of cardiopulmonary bypass (CPB; >or=2.4 L/min/m(2)) is a standard strategy used at this institute for children with pulmonary atresia (PA) due to a fear that the blood flow may be diverted by the major/minor aortopulmonary-collateral-arteries and hypervascularization due to long-term hypoxia. The purpose of this study was to describe the validity of high-flow management in children with PA. The CPB records of 23 children with PA who underwent a definitive biventricular repair between Feb 2006 and Nov 2008 were retrospectively reviewed. The mean age at the operation was 33 +/- 22 months. The blood-pressure during bypass was controlled with the same protocol. The mean cooling-temperature was 28.4 +/- 3.7 degrees C. The mean minimum hematocrit was 25.0 +/- 3.4%. The mean maximum bypass flow index at the initiation, the mean maximum flow index during aortic cross-clamping, the mean minimum flow index during aortic cross-clamping, and the mean maximum flow index after rewarming were 3.1 +/- 0.5, 3.1 +/- 0.5, 2.6 +/- 0.4, and 3.2 +/- 0.4 L/min/m(2), respectively. The higher bypass flow indexes significantly correlated with the lower serum lactate levels. The lowest oxygen delivery during CPB had significant influences on the urine output during bypass (R = 0.547, P = 0.007), the serum lactate levels at the end of CPB (R = -0.442, P = 0.035), and the postoperative thoracic effusion (R = -0.459, P = 0.028). A bypass flow index of 2.4 L/min/m(2) may not be sufficient and the maximum requirement of bypass flow index may be 3.2 L/min/m(2) or more in this patient population.

DOI： 10.1111/j.1525-1594.2009.00895.x

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The cognitive impairment and hemodynamic instability after neonatal cardiac surgery with cardiopulmonary bypass (CPB) might be exacerbated by hemodilution. Therefore, this study investigated the impact of different bloodless prime volumes on the hemodynamics and the inflammatory response by a miniaturized CPB system in neonatal piglets. The bypass circuit consisted of a Capiox RX05 (Capiox Baby RX, Terumo Corp., Tokyo, Japan) oxygenator and 3/16 internal diameter arterial and venous polyvinyl chloride tubing lines, with a minimum 75 mL prime volume. Twelve 1-week-old piglets were placed on a mild hypothermic CPB (32 degrees C) at 120 mL/kg/min for 2 h. The animals were divided into two groups, based on the volume of the prime solution. The priming volume was 75 mL in Group I and 175 mL in Group II. No blood transfusions were performed, and no inotropic or vasoactive drugs were used. The interleukin-6 (IL-6) and thrombin-antithrombin (TAT) complex levels, as well as right ventricular and pulmonary functions, were measured before and after CPB. Group I had low levels of IL-6 and TAT immediately after CPB (4370 +/- 2346 vs. 9058 +/- 2307 pg/mL, P < 0.01 and 9.9 +/- 7.7 vs. 25.1 +/- 8.8 ng/mL, P < 0.01, respectively). Group I had significantly improved cardiopulmonary function, cardiac index (0.22 +/- 0.03 vs. 0.11 +/- 0.05 L/kg/min, P < 0.001), and pulmonary vascular resistance index (7366 +/- 2860 vs. 28 620 +/- 15 552 dynes/cm(5)/kg, P < 0.01) compared with Group II. The miniaturized bloodless prime circuit for neonatal CPB demonstrated that the influence of hemodilution can reduce the subsequent inflammatory response. In addition, a low prime volume could therefore be particularly effective for attenuating pulmonary vascular resistance and right ventricular dysfunction in neonates.

DOI： 10.1111/j.1525-1594.2009.00922.x

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Language：Japanese   Publisher：膜型人工肺研究会  

1997年1月から2008年5月に実施された3100例の先天性心疾患手術症例の内、術後にExtracorporeal Membrane Oxygenation(ECMO)を導入した54例を生存群(20例)と非生存群(34例)に分け、月齢、身長、体重、体表面積、ECMO灌流量、ECMO補助期間、心臓手術中の体外循環時間、大動脈遮断時間を比較検討した。更に、ECMO補助期間についてNorwood手術後症例とその他の群、単心室形態症例群と両心室形態症例群において、生存群と非生存群に分けて多重比較による追加検討を行った。小児心臓ECMOに対する危険因子を分析した結果、低体重・低年齢の身体の小さな患者、ECMO導入期間、Norwood術後、単心室形態を持つ症例がECMO離脱困難症例であった。

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OBJECTIVE: Although a staged Fontan strategy allows for an excellent outcome in high-risk patients, an impaired ventricular function remains a significant factor of early/late mortality and morbidity. This study evaluated the clinical outcome of the Fontan operation in patients with impaired ventricular function. METHODS: A retrospective review was performed on 217 patients who had undergone the Fontan operation between 1991 and 2007. RESULTS: Twenty-nine (13%) of the 217 patients had an impaired ventricular function (ejection fraction (EF) <50%). The median age at the time of the operation was 3 (range: 1-31 years) years. There were five adult patients. The ventricular morphology was right in 20 patients (including five hypoplastic left heart syndrome (HLHS)) and others (left and two-ventricle) in nine patients. Heterotaxy syndrome was present in eight patients. Previous surgical interventions included bidirectional Glenn anastomoses in 24, modified Blalock-Taussig shunts in two and pulmonary artery banding in two. The preoperative EF was 43+/-6%. Significant (moderate or severe) atrioventricular valve regurgitation was noted in four patients. The percutaneous oxygen saturation (SpO(2)) was 82+/-5%. The pulmonary artery pressure and pulmonary artery index were 11+/-3 mmHg and 296+/-102 mm(2)m(-2), respectively. All 29 patients underwent the Fontan operation without any early mortality. There were two late mortalities and two re-operations. EF was maintained at 59+/-15% at a median follow-up of 7.5 (range: 1-19) years. The percent normal systemic ventricular end-diastolic volume decreased from 174+/-95% to 124+/-39% (p<0.05). The SpO(2) increased to 92+/-2%. The mean cardiothoracic ratio in chest X-ray and B-type natriuretic peptide were 51% (range: 35-68%) and 22 pgml(-1) (range: 9-382 pgml(-1)), respectively. Three patients developed congestive heart failure, seven had arrhythmia and two developed protein-losing enteropathy. The New York Heart Association (NYHA) class functional class is I in 21 patients, II in five and III in one. CONCLUSIONS: Acceptable clinical outcomes were observed at an intermediate follow-up of the Fontan operation in patients with an impaired ventricular function.

DOI： 10.1016/j.ejcts.2009.04.042

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Language：Japanese   Publisher：(公社)日本臨床工学技士会  

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Language：Japanese   Publisher：(公社)日本臨床工学技士会  

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We describe successful primary repair of 2 cases of transposition complex with aortic arch obstruction. A new aortic arch was reconstructed by direct anastomosis between the well-mobilized ascending aorta and the descending aorta. The neoaortic root with transferred coronary arteries was subsequently anastomosed to the undersurface of this new aortic arch. This technique deals with the significant size discrepancy between the 2 great arteries, and anomalous coronary artery patterns.

DOI： 10.1177/0218492309338126

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：MOSBY-ELSEVIER  

OBJECTIVE: The aim of this study was to evaluate right and left ventricular functions in patients with pulmonary arterial hypertension after living-donor lobar lung transplantation compared with those without hypertension. METHODS: Thirty-three recipients of living-donor lobar lung transplantation were divided into two groups: those with pulmonary arterial hypertension (PAH group; n = 12) and those without (non-PAH group; n = 21). Their systolic pulmonary artery pressure was 93.1 +/- 6.7 mm Hg versus 31.4 +/- 2.9 mm Hg, respectively. Right and left ventricular ejection fractions, systolic pulmonary artery pressure, and cardiac index were serially measured by radionuclide ventriculography and right heart catheterization, respectively. RESULTS: Pretransplant right and left ventricular ejection fractions were lower in the PAH group (29.8% +/- 7.0%, 49.9% +/- 6.6%) than in the non-PAH group (49.7% +/- 3.3%, 65.2% +/- 1.9%) (P = .010, .068). Two months after living-donor lobar lung transplantation, right ventricular ejection fraction and systolic pulmonary artery pressure in the PAH group (57.3% +/- 5.1%, 25.7 +/- 1.8 mm Hg) improved dramatically, equal to those in the non-PAH group. In contrast, left ventricular ejection fraction and cardiac index in the PAH group (50.9% +/- 3.7%, 2.66 +/- 0.12 L x min(-1) x m(-2)) were still significantly lower than in the non-PAH group (65.4% +/- 2.8%, 3.13 +/- 0.15 L x min(-1) x m(-2)) (P = .0038, .037). At 6 to 12 months, the PAH group demonstrated a significant rise in left ventricular ejection fraction and cardiac index that reached similar values in the non-PAH group measured at 2 months. These values were stable for up to 3 years. CONCLUSIONS: Right ventricular function recovered early after living-donor lobar lung transplantation in the PAH group. In contrast, recovery of left ventricular function required 6 to 12 months. Improved cardiac function was sustained for up to 3 years, suggesting long-term durability of cardiac function recovery after living-donor lobar lung transplantation.

DOI： 10.1016/j.jtcvs.2009.02.038

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Language：Japanese   Publisher：(NPO)日本心臓血管外科学会  

症例は5ヵ月女児。先天性副腎皮質機能不全にて近医で副腎皮質ホルモン補充療法を行っている。出生時より心雑音を指摘され近医転院、心室中隔欠損症の診断にて薬物療法を行っていた。生後4ヵ月までに心不全の状態は改善し、生後5ヵ月の時点で心カテーテル検査を施行した結果、肺体血流比2.9と高値であり、心不全の急性増悪をきたす恐れが強く、哺乳量が増えず体重増加があまり期待できないこと、易感染性であることから、早期に手術すべきと判断され、当院紹介となった。手術は心室中隔欠損症に対するパッチ閉鎖術を行った。周術期の糖質コルチコイド補充量は体表面積から投与ステロイド量を推定し、周術期の血中コルチゾール濃度の経時的変化を参考にしながら投与することで、良好な結果を得ることができた。乳幼児における開心術における糖質コルチコイド補充療法の報告は少なく、体表面積から投与量を推定することで良好な周術期管理を行うことができた。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2009&ichushi_jid=J01122&link_issn=&doc_id=20090724250010&doc_link_id=10.4326%2Fjjcvs.38.276&url=https%3A%2F%2Fdoi.org%2F10.4326%2Fjjcvs.38.276&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1007/s12574-009-0006-4

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A full-term newborn male infant presented with dyspnea and cleft lip and palate. He was thought to have esophageal atresia with tracheoesophageal fistula. He underwent bronchoscopy before operation that showed a laryngotracheoesophageal cleft (LTEC) type III. The left main bronchus originated from the lower esophagus. His diagnosis was communicating bronchopulmonary foregut malformation (CBPFM) type IA associated with LTEC type III. Enhanced chest computed tomographic scan showed the left pulmonary artery originated from the descending aorta. Staged operations were indicated. At first, reconstruction of the left pulmonary artery was done at 3 months of age. Then at 6 months of age, operations for LTEC (tracheoplasty and esophagostomy) and CBPFM left bronchoplasty were performed. Reconstruction of esophagus was performed at age of 1 year. He is now 3 years old and doing well with a mild degree of bronchomalacia. This is the first report of total reconstruction of CBPFM type IA associated with LTEC.

DOI： 10.1016/j.jpedsurg.2009.02.061

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Blood priming is necessary for cardiopulmonary bypass (CPB) in neonates to avoid excessive hemodilution
however, transfusion-related inflammation affects postCPB outcomes in neonatal open-heart surgery. We hypothesized that ultrafiltration of priming blood before CPB may reduce inflammatory mediators in priming blood and postCPB inflammatory responses, thereby improving cardiopulmonary function. Twelve 1-week-old piglets (3.5 ± 0.2 kg) were divided into two groups. Group U (n = 6) employed the priming blood ultrafiltrated before CPB, but group N (n = 6) used the nonultrafiltrated blood. Cardiopulmonary bypass was performed for 2 hours and then modified ultrafiltration (MUF) was conducted. Data were acquired before CPB and after MUF. The values of K, serotonin, and IL-8 in priming blood was significantly decreased after ultrafiltration (8.2 ± 2.6 vs. 4.2 ± 0.8 mEq/L, p &lt
0.01, 234 ± 96 vs. 74 ± 42 ng/ml, p &lt
0.01, 78.4 ± 5.1 vs. 64.5 ± 59.1 pg/ml, p &lt
0.05). Group U after MUF had lower thrombin-antithrombin complex levels (23.9 ± 5.1 vs. 33.7 ± 4.6 ng/ml, p &lt
0.01) and lower IL-8 levels in airway fluid (925 ± 710 vs. 2495 ± 1207 pg/ml, p &lt
0.05) than group N. Cardiac output and arterial PO2 after MUF in group U were also higher (1.13 ± 0.21 vs. 0.69 ± 0.22, p &lt
0.01, 340 ± 190 vs. 149 ± 84 mm Hg, p &lt
0.05). The ultrafiltration of blood priming before CPB attenuated activation of the coagulation pathway and inflammatory responses and preserved cardiopulmonary function in neonatal piglets. ©2009Amercian Society of Artificial Internal Organs.

DOI： 10.1097/MAT.0b013e31819b00c2

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This retrospective study analyzed the current practice of blood transfusion-free open-heart surgery in 536 children weighing 5-20 kg undergoing surgery between 2004 and 2007. A miniaturized cardiopulmonary bypass (CPB) circuit was used (priming volume
300 ml for the flow rate &lt
1,500 ml/min
550 ml for the flow rate of 1500-2300 ml/min). Modified ultrafiltration was routinely performed. Criteria for blood transfusion during CPB included a hematocrit of &lt
20% and/or mixed venous oxygen saturation of &lt
65%. Transfusion during CPB was avoided in 264 (49.3%) of the 536 patients (5-10 kg group, 29.0%
11-15 kg group, 67.4%
16-20 kg group, 80.8%). There was no neurological complication related to hemodilution. Multiple logistic regression analysis revealed that body weight, preoperative hematocrit, priming volume of CPB circuit, CPB time, and lowest hematocrit during CPB predict requirement of blood transfusion (p &lt
0.01). Transfusion rate was lowest in the atrial septal defect group (5.6%) and highest in tetralogy of Fallot group (78.7%), being associated with complexity of diagnosis and procedure required. Blood transfusion-free open-heart surgery may be achieved in the half of the patients weighing 5-20 kg, and further miniaturization of CPB circuit and refinement of perfusion strategy might reduce transfusion rate in patients &lt
10 kg and/or with complex congenital heart disease. ©2009Amercian Society of Artificial Internal Organs.

DOI： 10.1097/MAT.0b013e31819742f0

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BACKGROUND: Adult patients who do not fulfill the classical Fontan criteria now undergo total cavopulmonary connection (TCPC). However, limited information is available on the results for high-risk adult TCPC. METHODS: Twenty-five consecutive adult patients (aged 16 years or more) who underwent TCPC were retrospectively reviewed. The mean age at operation was 27 +/- 9 years (range, 16 to 52). The following items were considered as the potential risk factors according to previous reports: (1) aged more than 30 years (7 of 25); (2) heterotaxy (9 of 25); (3) systemic ventricular ejection fraction less than 50% (6 of 25); (4) atrioventricular valve regurgitation moderate or greater (6 of 25); (5) pulmonary arterial index less than 200 (7 of 25); (6) mean pulmonary arterial pressure 15 mm Hg or greater (3 of 25); (7) pulmonary arterial resistance 2.0 wood units or greater (11 of 25); (8) arrhythmias (13 of 25); (9) protein-losing enteropathy (3 of 25); (10) New York Heart Association (NYHA) functional class III or greater (9 of 25); (11) previous Fontan procedure (10 of 25); (12) systemic ventricular outflow obstruction (1 of 25); and (13) end-diastolic pressure of the systemic ventricle 11 mm Hg or higher (4 of 25). RESULTS: The mean follow-up period was 57 +/- 45 months (range, 0 to 154). All patients had at least 2 risk factors (range, 2 to 8). There was 1 early death and 2 late deaths. Comparing the late survivors and nonsurvivors, no statistical significance was identified in the above risk factors. However, the patients with 6 or more risk factors had a significantly higher mortality rate than patients with fewer than 6 risk risk factors (p < 0.01). Age (p = 0.08), NYHA class (p = 0.13), and protein-losing enteropathy (p = 0.08) may be risk factors for late death. CONCLUSIONS: The majority of the adult TCPC candidates tolerated the TCPC procedure in the early postoperative period. However, the accumulation of risk factors influences late mortality.

DOI： 10.1016/j.athoracsur.2008.10.040

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BACKGROUND: The purpose of this study was to describe the experience with staged surgical reconstruction of the hypoplastic left heart syndrome (HLHS) with a right ventricle to pulmonary artery conduit and to identify the risk factors that influence late outcome. METHODS: Between February 1998 and June 2007, 62 patients with HLHS underwent a Norwood procedure by using right ventricle to pulmonary artery conduit (median age, 9 days [range, 1 to 57]; median body weight 2.7 kg [range, 1.6 to 3.9 kg]). The subsequent 47 patients underwent a bidirectional Glenn procedure (stage 2). Thirty-two patients underwent a modified Fontan procedure (stage 3). Follow-up was complete (median, 32 months; range, 1 to 101). RESULTS: Hospital mortality after the Norwood procedure was 8% (5 of 62 patients). Between stages, 9 patients died, 3 before stage 2 and 6 before stage 3. There was 1 late death after stage 3. Overall survival was 76% (47 of 62). The estimated 1-year and and 5-year survival rates were 80% and 73%, respectively. Using the any-mortality as the endpoint, prematurity (gestational age <37 weeks), body weight less than 2.5 kg at stage 1 operation, and tricuspid regurgitation 2+ or more were associated with mortality. Using Cox regression analysis, body weight less than 2.5 kg and tricuspid regurgitation 2+ or more were two independent factors associated with midterm survival. CONCLUSIONS: From 9 years of experience, despite good early survival after Norwood stage 1 palliation, low body weight and tricuspid valve regurgitation were still associated with worse outcome. More efforts should be made to improve the late results for patients with hypoplastic left heart syndrome.

DOI： 10.1016/j.athoracsur.2008.08.027

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BACKGROUND: The purpose of the present study was to evaluate the effect of modified ultrafiltration (MUF) on neonates with transposition of the great arteries (TGA) undergoing arterial switch operation. METHODS AND RESULTS: The current study included 36 neonates who underwent an arterial switch operation between 1998 and 2006. Arterio-venous MUF was done in 15 patients (MUF-treated group) and the other 21 patients were controls. Parameters included hematocrit, hemodynamics, pulmonary function, drain loss, leak of peritoneal fluid, length of intubation, and intensive care unit (ICU) stay. The hematocrit increased from 34+/-2% to 47+/-4% in the MUF-treated group. Blood pressure in the MUF-treated group was significantly increased without any change of central venous or left atrial pressure. Post-operative oxygenation in the MUF-treated group was greater than that of the control group (P/F ratio: 258+/-92 vs 170+/-100 mmHg, p<0.05), which did not contribute to decrease in intubation time (54+/-33 vs 52+/-29 h, p=NS). Post-operative chest drain loss and peritoneal fluid leak were comparable. The ICU stay in the MUF-treated group was significantly shorter than that in the controls (101+/-34 vs 139+/-42 h, p<0.05). CONCLUSIONS: MUF brought improvement in blood pressure and gas exchange capacity and subsequent shorter ICU stay. MUF did not have significant impact on intubation time and capillary leak.

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Language：Japanese   Publishing type：Research paper (scientific journal)  

The arterial switch operation (ASO) has become the primary surgical approach used for correction of transposition of the great arteries. All the prerequisites for a successful ASO were recognized in time and dealt with, which allowed general acceptation of the technique. We report on our technique for the procedure and the result to date. From January 1991 to January 2008, a total of 100 patients underwent ASO at our unit using medially-based trapdoor flap method. The neo-pulmonary artery (PA) was reconstructed using a single rectangular pericardial patch. The initial patient having intramural coronary artery died due to ischemic event after Aubert procedure. Three patients had re-right ventricular out flow tract repair (RVOTR) in a long-term follow-up period. There was no significant aortic insufficiency, no ischemic event and no lethal arrhythmia.

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Language：Japanese   Publisher：(一社)日本不整脈心電学会  

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A 35-year-old man with a history of total correction of tetralogy of Fallot (TOF) fell down while riding a bike and experienced blunt cardiac rupture. His vital signs were stable because the bleeding was limited by an adhesion caused by the previous operation. Chest computed tomography clearly displayed the ruptured points, and an emergency operation was performed. Because a pneumothorax was suspected, a cardiopulmonary bypass was established with a femorofemoral bypass while the patient was conscious before artificial ventilation was initiated. Two ruptured points were detected on the anterior wall of the right ventricle and were repaired by suturing. The patient recovered and was discharged without any major complications 40 days after the operation. This is the first published case of blunt cardiac rupture after total correction of TOF.

DOI： 10.1007/s11748-007-0206-7

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

This study was undertaken to determine the impact of miniaturization of a cardiopulmonary bypass (CPB) circuit on blood transfusion and hemodynamics in neonatal open-heart surgery. Neonates (n = 102) undergoing open-heart surgery between 2002 and 2006 were included and divided into three groups: group 1 (n = 28), Dideco 902 oxygenator + 5/16 '' line; group 2 (n = 29), Dideco 901 oxygenator + 1/4 '' line; group3 (n = 45), Dideco 901 oxygenator + 3/16 '' arterial + 1/4 '' venous line. Amount of priming volume, blood and bicarbonate sodium use during CPB, and hemodynamics were compared. Priming volume in the groups 2 and 3 was significantly less compared with the group 1 (group 1, 575 37 ml; group 2, 328 +/- 12 ml, group 3, 326 +/- 5 ml, p < 0.05). Blood transfusion and bicarbonate sodium use during CPB in groups 2 and 3 were significantly less compared with group 1. Hemodynamics during CPB was comparable. There were no differences between groups 2 and 3 in any parameter. Miniaturization of the CPB circuit resulted in decrease in priming volume and subsequent reduction in blood and bicarbonate sodium use. Downsizing the lines had minimal impact on any of the parameters studied, and further efforts should be made to achieve neonatal open-heart surgery without blood transfusion.

DOI： 10.1097/MAT.0b013e318158ddf6

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Language：Japanese   Publishing type：Research paper (scientific journal)  

PubMed

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVES: The aim of this study was to evaluate the long-term effects of living-donor lobar lung transplantation (LDLLT) for critically ill patients with pulmonary arterial hypertension (PAH) who failed in epoprostenol treatment. BACKGROUND: Although continuous epoprostenol infusion has markedly improved survival in patients with PAH, some patients do not benefit from this therapy. METHODS: From July 1998 to December 2003, 28 consecutive PAH patients who were treated with epoprostenol and accepted as candidates for lung transplantation were enrolled. All data were prospectively collected. As of July 2006, LDLLT was performed in 11 of those patients whose condition was deteriorating. Cadaveric lung transplantation (CLT) was performed in 2 patients. Medical treatment was continued in 15 patients. RESULTS: There was no mortality in patients receiving LDLLT during a follow-up period of 11 to 66 months (average 48 months), and all patients returned to World Health Organization functional class I. Mean pulmonary artery pressure decreased from 62 +/- 4 mm Hg to 15 +/- 2 mm Hg (p < 0.001) at discharge and remained normal at 3 years. One CLT patient died of primary graft failure. Among medically treated patients, 6 patients died of disease progression. The survival rate was 100% at 5 years for patients receiving LDLLT, and 80% at 1 year, 67% at 3 years, and 53% at 5 years for patients medically treated (p = 0.028). All living donors have returned to their previous lifestyles. CONCLUSIONS: These follow-up data support the option of LDLLT in patients with PAH who would die soon otherwise.

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Language：English   Publishing type：Research paper (scientific journal)  

We describe a successful case of surgical treatment for anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome with severe left ventricular dysfunction. Because of the severe left ventricular dysfunction, we planned to use an extracorporeal membrane oxygenation for heart support until a satisfactory recovery had been established. The left ventricular function significantly recovered in a few days, and the patient could be discharged without any complications.

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Language：Japanese   Publisher：膜型人工肺研究会  

症例は3ヵ月女児で、高度左室機能低下・僧帽弁逆流を伴う左冠状動脈肺動脈起始異常症と診断された。胸部X線写真は、心胸比70%と右無気肺を示した。心電図で洞性頻脈、軽度ST低下、陰性T波、異常Q波、左室肥大を認めた。左冠状動脈は造影されなかった。経食道心臓超音波検査では、駆出率:測定不能、短縮率:10%、高度〜中等度僧帽弁逆流、左室中隔の壁運動低下がみられた。心房間交通を作成し、大動脈遮断解除後、約30分間の左心補助を行い、右房脱血、上行大動脈送血によるV-A extracorporeal membrane oxygenation(ECMO)へ移行継続した。カテコラミン・血管拡張剤の増量、ECMO補助流量の段階的減量により術後7日目に人工呼吸器から離脱し、短縮率・僧帽弁逆流は改善傾向となり、心胸比も縮小した。修復術後に肺うっ血や肺高血圧の可能性がある症例への心房間交通の作成とECMOを組み合わせた機械的循環補助は、有効であると思われた。

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Language：English   Publishing type：Research paper (scientific journal)  

PubMed

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

Single-ventricle palliation without the use of cardiopulmonary bypass carries advantages that reduce systemic edema and inflammatory responses; however, simple clamping of the superior vena cava (SVC) without a temporary shunt leads to increase in cerebral venous pressure and subsequent decrease in cerebral blood flow during bidirectional cavopulmonary shunt (BCPS). We report our experience of BCPS, using a centrifugal pump-assisted temporary shunt.The criteria included an unrestrictive interatrial communication, the absence of atrioventricular valve regurgitation, and the existence of an antegrade pulmonary blood flow. From August 2000, 14 children with single-ventricle physiology met the criteria. The mean age was 1.0 +/- 0.9 years, and the mean weight was 8.4 +/- 2.6 kg. A temporary shunt was established between the SVC and the right atrium with right-angle cannulae, which were connected to a centrifugal pump to accelerate the blood flow from the SVC to the right atrium.All patients tolerated the procedure. Mean central venous pressure was 17 +/- 4 mm Hg, and transcutaneous oxygen saturation was maintained at 77 +/- 8% during anastomosis. No patients required blood transfusion. There were no postoperative neurological complications.The centrifugal pump-assisted temporary shunt offered safer and more effective circulatory support than other shunt systems, with excellent venous drainage in pediatric patients undergoing BCPS.

DOI： 10.1097/01.mat.0000235454.64316.f3

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

To minimize myocardial ischemia, we repaired aortic arch obstruction with ventricular septal defect, using two different techniques of cerebral and myocardial perfusion.Seventy-one infants, ages 3 to 137 days, underwent primary repair of coarctation of the aorta (n = 49)/interruption of the aortic arch (n = 22) with ventricular septal defect. In 65 patients, an end-to-end arch anastomosis was performed with cerebral and myocardial perfusion through the innominate or the ascending arterial cannula (non-working beating heart: NWBH). In the remaining 6 patients, an arterial cannula was placed into the innominate artery. With partial cardiopulmonary bypass, the innominate artery was snared proximal to the cannulation site and the ascending aorta was cross-clamped. An extended arch anastomosis was carried out with cerebral perfusion and a working beating heart (WBH).Ten patients (15%) undergoing aortic arch repair with the NWBH technique required cardioplegic arrest to complete a proximal anastomosis, whereas in all 6 repairs with the WBH technique, the extended anastomoses were completed without myocardial ischemia. One hospital death and late death occurred, with an overall survival of 98%.End-to-end arch reconstruction is feasible without myocardial ischemia, using the NWBH technique in patients without hypoplastic arch and using the WBH technique in patients with hypoplastic arch.

DOI： 10.1097/01.mat.0000235276.77489.bb

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BACKGROUND: Patients with double inlet left ventricle/l-transposition and similar morphologies have their systemic outflow traverse a bulboventricular foramen (BVF), which has a propensity to narrow over time. A Norwood procedure may be performed as the initial palliation. We prefer aortic arch repair and pulmonary artery banding, delaying Damus-Kaye-Stansel (DKS) or BVF resection until the second palliation. The aims of this study were to compare our results with those reported for Norwood strategy and examine the development of systemic outflow obstruction. METHODS: Retrospective study of patients with double inlet left ventricle, L-TGA or similar morphology presenting between 1990 and 2000. Follow-up with clinical assessment, echocardiography and catheter studies. RESULTS: Twenty-five patients had initial palliation with pulmonary artery banding with repair of any associated arch obstruction. Twelve patients had DKS performed as part of their second stage procedure, and 3 had DKS performed later for recurrent stenosis after prior enlargement of BVF. Six patients had BVF resection without later restenosis and 4 patients did not develop BVF stenosis. There was one early death (4%) and two late (8%). Fontan completion was achieved in 20 of the 22 survivors. There were no cases of DKS obstruction, no pulmonary valve had more than mild regurgitation. CONCLUSIONS: Our approach achieves low operative mortality and morbidity and compares favorably with reported results for Norwood palliation. The significant rate of systemic outflow obstruction in those who did not undergo DKS at the second stage confirms the utility of early DKS in children with this morphology.

PubMed

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OBJECTIVE: Pulmonary overcirculation through a systemic-pulmonary shunt has been one of the major causes of early death after the Norwood procedure. To avoid this lethal complication, we constructed a right ventricle-pulmonary shunt in first-stage palliation of hypoplastic left heart syndrome. METHODS: Between February 1998 and February 2002, 19 consecutive infants, aged 6 to 57 days (median, 9 days) and weighing 1.6 to 3.9 kg (median, 3.0 kg), underwent a modified Norwood operation with the right ventricle-pulmonary artery shunt. The procedure included aortic reconstruction by direct anastomosis of the proximal main pulmonary artery and a nonvalved polytetrafluoroethylene shunt between a small right ventriculotomy and a distal stump of the main pulmonary artery. The size of the shunt used was 4 mm in 5 patients and 5 mm in 14. RESULTS: All patients were managed without any particular manipulation to control pulmonary vascular resistance. There were 17 survivors (89%), including 3 patients weighing less than 2 kg. Two late deaths occurred due to obstruction of the right ventricle-pulmonary artery shunt. Thirteen patients underwent a stage II Glenn procedure after a mean interval of 6 months, with 2 hospital deaths. To date, a stage III Fontan procedure has been completed in 4 patients. Overall survival was 62% (13/19). Right ventricular fractional shortening at the last follow-up (3-48 months after stage I) ranged from 26% to 43% (n = 13, mean, 33%). CONCLUSION: Without delicate postoperative management to control pulmonary vascular resistance, the modified Norwood procedure using the right ventricle-pulmonary shunt provides a stable systemic circulation as well as adequate pulmonary blood flow. This novel operation may be particularly beneficial to low-birth-weight infants with hypoplastic left heart syndrome.

PubMed

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PubMed

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OBJECTIVE: To prevent possible deleterious effects of right ventricular volume overload on cardiorespiratory function, we developed a total right ventricular exclusion procedure for the treatment of end-stage isolated congestive right ventricular failure. METHODS: Since 1996, this procedure has been performed in 5 patients in New York Heart Association functional class IV: 2 adults with arrhythmogenic right ventricular dysplasia and 3 children with Ebstein anomaly. The entire right ventricular free wall was resected along the atrioventricular groove and then parallel to the interventricular septum, sparing the pulmonary valve and a skeletonized right coronary artery. The orifice of the tricuspid valve was closed with either a polytetrafluoroethylene patch or with its leaflets. The defect of the right ventricular free wall was covered with a polytetrafluoroethylene patch in the 2 patients with arrhythmogenic right ventricular dysplasia and directly closed with the remnant of the free wall in the 3 children with Ebstein anomaly. After resection of a redundant right atrial wall, coronary sinus blood flow was rerouted into the left atrium through an atrial septal defect. A total cavopulmonary connection was constructed in 4 patients and a bidirectional superior cavopulmonary anastomosis in 1 infant. The heart was controlled with a DDD pacemaker in 3 patients. RESULTS: The patients were extubated at a mean of 14 hours postoperatively (range, 1-38 hours). There were no early or late deaths. At follow-up, ranging from 8 to 57 months, the mean cardiothoracic ratio had decreased from 74% +/- 7% before the operation to 52% +/- 6% (P <.01). All patients are in functional class I. Neither of the patients with arrhythmogenic right ventricular dysplasia have had attacks of ventricular tachycardia nor are they using antiarrhythmic medication. CONCLUSIONS: The total right ventricular exclusion procedure provides effective decompression of the lung, as well as the left ventricle, and may result in more effective volume loading of a surgically created single ventricle with increased systemic output. We believe that this new surgical option offers rescue treatment for isolated end-stage right ventricular failure in critically ill patients.

PubMed

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Language：English   Publisher：International Heart Journal Association  

Five hundred and sixty-one endocardial pacing leads implanted in the right ventricle of 502 patients between 1971 and 1990 were followed for up to 17 years regarding their complications, stimulation threshold behavior, and overall longevity.<br>Lead tip dislodgement occurred in 16 leads (2.9%), in 14 of which dislodgement occurred within 2 months after implantation. The incidence of dislodgement was significantly smaller in tined leads than in nontined leads. Lead conductor fracture occurred in 19 leads (3.4%), in 15 of which fracture occurred within 5 years after implantation. Two particular sites of lead fracture were identified; i.e., one within the pacemaker pocket and the other at a particular point in the subclavian vein between the clavicle and the first rib. Fixation ligature in the former site and the venopuncture point for lead insertion in the latter site are thought to be related to lead fracture.<br>Other complications included insulator break in 3 leads (0.5%), exit block in 7 leads (1.2%), and poor sensing in 2 leads (0.4%).<br>The cumulative survival of leads was 94.1±2.7% (mean±95% confidence interval) at 5 years, 86.3±6.3% at 10 years, and 74.2±14.0% at 15 years after implantation.<br>The minimal stimulation threshold, lead impedance and R wave amplitude were all found to be stable for up to 10 years, and there were no tendencies toward higher stimulation threshold during the observation period of this study.

DOI： 10.1536/ihj.34.193

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Responsible for pages：297-299  

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Responsible for pages：886-892  

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Language：Japanese   Publisher：(一社)日本外科学会  

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Language：Japanese   Publisher：(一社)日本外科学会  

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Language：Japanese   Publisher：(株)南江堂  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2020&ichushi_jid=J00349&link_issn=&doc_id=20201001110013&doc_link_id=issn%3D0021-5252%26volume%3D73%26issue%3D10%26spage%3D789&url=http%3A%2F%2Fwww.pieronline.jp%2Fopenurl%3Fissn%3D0021-5252%26volume%3D73%26issue%3D10%26spage%3D789&type=PierOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00005_2.gif

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Language：Japanese   Publisher：(株)南江堂  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J00349&link_issn=&doc_id=20191129180023&doc_link_id=10.15106%2Fj_kyobu72_1066&url=https%3A%2F%2Fdoi.org%2F10.15106%2Fj_kyobu72_1066&type=%88%E3%8F%91.jp_%83I%81%5B%83%8B%83A%83N%83Z%83X&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

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Language：Japanese   Publisher：(株)南江堂  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J00349&link_issn=&doc_id=20190411040010&doc_link_id=40021866169&url=http%3A%2F%2Fci.nii.ac.jp%2Fnaid%2F40021866169&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_1.gif

Language：Japanese   Publisher：(一社)日本外科学会  

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Language：Japanese   Publisher：(一社)日本集中治療医学会  

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Language：Japanese   Publisher：岡山医学会  

筆者らは高齢社会医療・介護機器研究推進講座を設立し、既存のインターネット回線を利用して、超小型の携帯心電計(CarPod)とマット型生体センサー(おだやかタイム)を用い、僻地、離島、災害地や在宅寝たきり老人の医療現場から訪問看護施設を通じ、主治医や専門医の在宅および遠隔医療が可能になるシステムを確立した。これらの取り組みを紹介し、生体情報リアルモニタリングの重要性と臨床応用について概説した。

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Language：Japanese   Publisher：日本臨床外科学会  

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Language：Japanese   Publisher：(株)南江堂  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2018&ichushi_jid=J00349&link_issn=&doc_id=20180830120021&doc_link_id=10.15106%2Fj_kyobu71_648&url=https%3A%2F%2Fdoi.org%2F10.15106%2Fj_kyobu71_648&type=%88%E3%8F%91.jp_%83I%81%5B%83%8B%83A%83N%83Z%83X&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

Language：Japanese   Publisher：(一社)日本てんかん学会  

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Language：Japanese   Publisher：岡山医学会  

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Language：Japanese   Publisher：(有)科学評論社  

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Language：Japanese   Publisher：若年者心疾患・生活習慣病対策協議会  

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Language：English   Publisher：日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Other Link： http://search.jamas.or.jp/link/ui/2017220959

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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J-GLOBAL

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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