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

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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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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OBJECTIVE: To evaluate the safety and efficacy of surgical repair for patients diagnosed with simple congenital heart defects (CHD) using the minimally invasive right vertical infra-axillary minithoracotomy (RVIAT) approach. METHODS: We retrospectively reviewed the clinical data of consecutive patients who underwent minimally invasive RVIAT for repair of CHD between August 2019 and August 2022. There were 382 patients who underwent 8 primary procedures and were included in this study. RESULTS: The median age of the patients was 16.2 (interquartile range [IQR], 7.2 to 41.9) months, and the median weight of the patients was 8.8 (IQR, 6.5 to 14) kg. The preoperative diagnoses were as follows: ventricular septal defect, atrial septal defect, partial anomalous pulmonary venous return, partial atrioventricular septal defect, cor triatriatum, complete atrioventricular septal defect, and myxoma. The mean aortic cross-clamp time, bypass time, and operation time were 45.4 ± 19.3 min, 65.6 ± 23.1 min, and 154.5 ± 29.7 min, respectively. There was no in-hospital mortality or conversion to median sternotomy. Two patients (0.5%) required early reoperation; 1 due to postoperative bleeding and 1 for permanent pacemaker implantation. Other complications included trivial residual shunts (23 of 382, 6%), pleural effusion (3 of 382, 0.8%), pneumothorax (0.8%), and wound infection (4 of 382, 1%). There were 2 late noncardiac deaths. Late reoperation was performed on 1 patient with progressive aortic valve regurgitation who required aortic valvuloplasty. CONCLUSIONS: RVIAT is a minimally invasive approach that can be safely performed on patients with simple CHDs. RVIAT may be a good alternative approach for median sternotomy and cardiac intervention.

DOI： 10.1177/15569845241273650

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DOI： 10.1007/s12328-023-01892-w

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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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OBJECTIVE: This study investigated the safety of performing surgical repair for doubly committed ventricular septal defects by right vertical infra-axillary minithoracotomy (RVIAT). METHODS: A retrospective comparative study was performed to evaluate the outcomes of patients who underwent doubly committed ventricular septal defects closure from January 2019 to May 2022. Seventy-four patients were enrolled in the study and treated with either the median sternotomy approach (MSA: n = 37) or the RVIAT approach (RVIAT: n = 37). RESULTS: The median weight and age in the MSA group were significantly lower than those in the RVIAT group (MSA: 6.0 kg [interquartile range] (IQR), 5.2 to 8.7 kg] vs RVIAT: 7.5 kg [IQR, 5.6-14 kg]; P = .034 and MSA: 4.9 months [IQR, 3.6-9.4 month] vs 9.6 months [IQR, 5.0-60.4 months]; P = .0084). No patients died, and no patients in the RVIAT group required conversion to the MSA approach. The mean prebypass surgical time was longer in the RVIAT group (36.1 ± 8.2 minutes vs 31.8 ± 5.6 minutes; P = .03). There were no significant differences between the 2 groups in cardiopulmonary bypass time, aortic crossclamp time, or operation time. Significantly shorter ventilation times were observed in the RVIAT group (11.9 ± 8.2 hours vs 15.4 ± 6.3 hours; P = .006). CONCLUSIONS: Closure of doubly committed ventricular septal defects through the pulmonary trunk by the RVIAT approach is feasible and safe, and does not increase the risk of bypass-related complications.

DOI： 10.1016/j.xjon.2023.06.012

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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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DOI： 10.1016/j.jtcvs.2023.07.040

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

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Abstract

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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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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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 <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 <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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Language：English   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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DOI： 10.1016/j.xjtc.2022.07.020

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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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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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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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The disadvantage of right ventricle-to-pulmonary artery (RV-PA) shunt is the need for more unplanned interventions to address stenosis in the shunt or branch pulmonary arteries, as compared to the modified Blalock-Taussig shunt group. Ring-enforced RV-PA PTFE conduit and dunk technique minimized these complications and right ventricle (RV) damage. Aortic arch obstruction increases afterload and leads to ventricular dysfunction and tricuspid regurgitation; therefore, most surgeons prefer to use homograft, autologous pericardium, or bovine pericardium to reconstruct the neoaorta. Artificial materials decrease the elastic properties, increase wall stiffness, and decrease the distensibility of the aorta; and as a result, RV function gradually deteriorates. This inelastic reconstructed aorta may be one of the reasons why long-term outcomes after the Fontan procedure are worse in hypoplastic left heart syndrome (HLHS) patients, in comparison to non-HLHS. Reconstruction of the neoaorta without any patch materials, or at least techniques that largely minimize the use of non-autologous materials, will offer a further refinement of our ability to optimize ventriculoarterial coupling and thereby long-term RV function.

DOI： 10.1177/21501351221116766

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DOI： 10.1016/j.xjtc.2022.02.037

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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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DOI： 10.1177/02184923221094958

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DOI： 10.1016/j.jtcvs.2021.08.083

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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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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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Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11 days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.

DOI： 10.1007/s00246-021-02782-z

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DOI： 10.1016/j.xjon.2021.08.024

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Surgical restoration of subvalvular geometry is very important to prevent recurrence of ischemic/functional mitral regurgitation (MR). 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 MR with minimal leaflet tethering and improved systolic LV function. This technique may be an aggressive and encouraged approach for patients with ischemic/functional MR.

DOI： 10.1016/j.athoracsur.2021.03.095

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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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DOI： 10.1053/j.optechstcvs.2022.03.003

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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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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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Aortic root abscess is one of the most catastrophic complications of aortic valve endocarditis. Complete débridement 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 aortomitral continuity extending to the commissure between the left and right coronary cusps. After débridement, the aortic annulus underneath the left coronary artery was reconstructed using a handmade aortomitral monobloc valve without aortic annuloplasty. This valve was required for the extensive root abscess of the left and noncoronary sinus to achieve complete débridement.

DOI： 10.1016/j.athoracsur.2020.11.046

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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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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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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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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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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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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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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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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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DOI： 10.1177/0218492318811557

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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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DOI： 10.1016/j.jcin.2018.06.055

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BACKGROUND: We hypothesized that chylothorax could be a sign of intolerance to the Fontan physiology, and thus patients who develop chylothorax or pleural effusion have worse medium-term to long-term survival. METHODS: A total of 324 patients who underwent the Fontan operation between 2000 and 2013 were included. Chylothorax was defined as ≥5 mL/kg/day of chylomicron-positive chest drainage fluid no earlier than postoperative day 5 or drainage with >80% lymphocytes. Outcomes were compared between the chylothorax and non-chylothorax groups by the Kaplan-Meier method and log-rank test. Independent predictors of chylothorax and number of days of any chest drainage were analyzed with multivariable logistic regression and multivariable generalized negative binomial regression for count data, respectively. RESULTS: Chylothorax occurred in 78 patients (24%). Compared with the non-chylothorax group, the chylothorax group had a longer duration of chest tube requirement (median, 18 days vs 9 days; P < .000) and a longer length of hospital stay (median, 19 days vs 10 days; P < .000). Eight patients (10.3%) required thoracic duct ligation. The chylothorax group had lower freedom from death (P = .013) and from composite adverse events (P = .021). No predictor was found for chylothorax. Pulmonary atresia (P = .031) and pre-Fontan pulmonary artery pressure (P = .01) were predictive of prolonged pleural effusion (>14 days). CONCLUSIONS: Occurrence of chylothorax following the Fontan operation can be a marker of poorer medium-term clinical outcomes. It is difficult to predict occurrence of chylothorax owing to its multifactorial nature and involvement of lymphatic compensatory capacity that is unmasked only after the Fontan operation.

DOI： 10.1016/j.jtcvs.2017.11.046

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BACKGROUND: We sought to evaluate the incidence of Fontan failure or complication and its relation to death in patients having contemporary Fontan strategies over 2 decades. METHODS: Five hundred patients who underwent Fontan completion (extracardiac, n = 326; lateral tunnel, n = 174) from 1985 to 2012 were reviewed. Patient characteristics, modes of Fontan failure/complication and death, and predictors for Fontan failure/complication and death were analyzed. RESULTS: There were 23 early deaths (4.6%) and 17 late deaths (3.4%), with no early death since 2000. Survival has improved over time (p < 0.001). Twenty-three of 40 patients who died were identified as Fontan failure before death, including ventricular dysfunction (n = 14), pulmonary vascular dysfunction (n = 4), thromboembolism (n = 2), and arrhythmia (n = 4). Mode of death was circulatory failure (n = 18), multiorgan failure (n = 6), pulmonary failure (n = 3), cerebral/renal (n = 5), and sudden death (n = 4). Modes of failure/complication were directly (65%) or conceivably (10%) related to death in 30 of 40 patients (75%). Forty-eight percent of survivors had late Fontan complication(s). Five-year freedom from late Fontan complication was lower among patients who died compared with patients who survived (29.4% versus 53.3%, p < 0.001). Ventricular dysfunction (p = 0.001) and higher pulmonary artery pressures (p < 0.001) after Fontan were predictors for death. Longer cardiopulmonary bypass time (p = 0.032) and reinterventions (p < 0.001) were predictors for late Fontan complication. CONCLUSIONS: Early death in the early era has been overcome. Yet the incidence and causes of late death remain unchanged. There was a strong causative relationship between the mode of Fontan failure/complication and death, indicating the importance of early recognition and treatment of Fontan failure/complication.

DOI： 10.1016/j.athoracsur.2017.10.047

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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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DOI： 10.1016/j.jtcvs.2017.09.018

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This report describes a case of nonbacterial thrombotic endocarditis caused by Waldenström macroglobulinemia, with diffuse endocardial lesions and involvement of all 4 cardiac valves. A 77-year-old man presented with heart failure due to severe regurgitation of all 4 cardiac valves; surgical repair using bioprosthetic valves was indicated. A pathological study revealed fibrin-triggered thrombus formation that confirmed the diagnosis of nonbacterial thrombotic endocarditis. In cases of nonbacterial thrombotic endocarditis, the underlying cause should be investigated.

DOI： 10.1177/0218492317748091

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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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Diagnosis of aortic dissection complicating cerebral ischemic stroke is difficult. A 60-year-old patient presented at an emergency department of a hospital with a sudden development of left-sided weakness, but without chest pain. During administration of recombinant tissue plasminogen activator with diagnosis of cerebral infarction, he developed symptomatic hypotension and was transferred to our hospital. His chest computed tomography revealed Stanford type A aortic dissection. Replacement of the ascending aorta and aortic arch was successfully performed on the 4th day.

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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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OBJECTIVE: The hybrid approach for hypoplastic left heart syndrome (HLHS) could theoretically result in better preservation of right ventricular (RV) function then the Norwood procedure. The aim of this study was to compare echocardiographic indices of RV size and function in patients after Norwood and hybrid throughout all stages of palliation. METHODS: 76 HLHS patients (42 Norwood, 34 hybrid) were retrospectively studied. Echocardiography was obtained before stage I, before and after stage II, and before and after Fontan. Median follow-up was 4.9 years (range 1.1-8.5). RESULTS: Baseline characteristics before stage I were similar. Hybrid patients demonstrated a significant decrease in RV fractional area change (FAC) between baseline and pre-stage II (36±9% vs 27±6%; p<0.01); Norwood patients remained stable (32±10% vs 32±7%; p=0.21). At pre-stage II, moderate/severe tricuspid valve (TV) regurgitation was found in nine Norwood (33%) and four hybrid (18%) patients (p=0.19). After stage II, the difference in FAC became insignificant (29±7% vs 25±8%, p=0.08) and moderate/severe TV regurgitation (TR) was found in 13 Norwood (48%) and four hybrid patients (19%) (p=0.18). At pre-Fontan, RV FAC was similar after Norwood and hybrid (34±5% vs 33±6%, p=0.69), which remained unchanged after Fontan. After Fontan, one Norwood and one hybrid patient had moderate TR. RV and TV size were similar for both groups at each time point. CONCLUSIONS: Patients after Norwood and hybrid procedures had equivalent indices of RV size, and systolic and diastolic function throughout all stages of palliation. Small differences in individual RV and TV indices are likely to be explained by differences in physiology or surgical timing rather than by intrinsic differences in myocardial and valve function.

DOI： 10.1136/heartjnl-2015-308787

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DOI： 10.1016/S0735-1097(16)30948-2

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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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Authorship：Lead author   Language：English   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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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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BACKGROUND: The purpose of this study was to evaluate the clinical utility of intraoperative aortic blood flow measurements on clinical outcome in patients undergoing pulmonary artery banding (PAB). METHODS: We reviewed 12 patients who underwent a PAB between September 2008 and March 2013 who also had intraoperative aortic blood flow measurements. Diagnosis included biventricular physiology in 6, single-ventricle physiology in 4, and inadequate systemic ventricle in 2 patients. Aortic blood flow was measured at the time of surgery by Transonic flow probe (Transonic Systems Inc, Ithaca, NY). Aortic flow, intraoperative hemodynamics, and clinical outcomes were analyzed to determine the potential predictive utility of intraoperative variables on postoperative outcomes. RESULTS: The aortic flow increased after PAB from 1.56 ± 0.73 to 2.20 ± 1.10 L · min(-1) · m(-2) (41.0% increase; p = 0.001). The efficacy of the PAB procedure was found to be directly related to the percentage increase in aortic blood flow measured intraoperatively. Three patients with less than 20% increase in aortic blood flow died, required re-PAB, or developed ventricular dysfunction, while patients with successful PAB had more than 40% increase in aortic blood flow. The percentage increase in aortic blood flow was not predictable based on pre-band or post-band absolute aortic blood flow measurements. The percentage increase in aortic blood flow was inversely correlated to the tightness of the PAB as defined relative to that predicted by the Trusler formula (r = 0.67; p = 0.01). CONCLUSIONS: This study identifies the change in the aortic blood flow as a new, physiologically based parameter to help predict PAB outcome.

DOI： 10.1016/j.athoracsur.2015.01.066

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BACKGROUND: Patients with single-ventricle physiology frequently develop left-sided pulmonary vein obstruction (PVO), in which the pulmonary veins traverse the descending thoracic aorta. We hypothesized that a combination of cardiomegaly and an anteriorly positioned descending aorta is associated with PVO. METHODS: Among 494 consecutive single-ventricle patients, 15 were diagnosed with PVO by cardiac magnetic resonance, defined as anatomically localized narrowing of the pulmonary vein diameter. Using axial slices at the level of the left lower pulmonary vein, normalized dimensions were obtained to characterize the anatomic relationships of intrathoracic structures. Measurements were compared between patients with PVO and "control" patients (single-ventricle patients with normal pulmonary veins, n = 12). RESULTS: Patients with cardiac magnetic resonance-diagnosed PVO had larger cardiac size and more antero-laterally located descending aorta when compared with controls (normalized dimensions: cardiac/thoracic area ratio: 0.43 vs 0.38, P = .035, distance from vertebra to descending aorta normalized by the horizontal dimension of thoracic cavity: 0.09 vs 0.08, P = .049). Seven (47%) patients underwent PV sutureless repair, and 3 (of 7) failed to achieve Fontan. Patients who failed to achieve Fontan had a larger normalized cardiac size than those who achieved Fontan (cardiac/thoracic area ratio: 0.49 vs 0.39, P = .001). CONCLUSIONS: The combination of relative cardiomegaly within the context of the thoracic cavity at the level of the pulmonary veins and antero-lateral displacement of the aorta is associated with left-sided PVO and subsequent failure to achieve Fontan completion. Further characterization of these unique geometric relationships may help inform both surveillance strategies and decision making in the timing of interventions, and guide the intraoperative objectives at the time of PVO repair.

DOI： 10.1016/j.jtcvs.2014.11.089

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BACKGROUND: We sought to evaluate the medium-term implications of fenestration status. METHODS: Between 1994 and 2012, 326 patients received an extracardiac Fontan (hospital mortality n = 6, 1.8%). A fenestration was routinely created (n = 306, 94%) unless there was technical difficulty. Three hundred patients discharged with an open fenestration were included. The primary end points were death and Fontan failure. Secondary outcomes were Fontan complications such as venovenous collaterals, protein-losing enteropathy, pacemaker requirement, and arrhythmias. RESULTS: The fenestration was closed in 260 patients: 185 as a catheter intervention (62%) and 75 (25%) spontaneously. Forty patients (13%) had the fenestration open at a median follow-up period of 5.05 years. Of these patients, catheter-based closure failed in 10 (3%). There was no statistically significant difference in pre-Fontan hemodynamic parameters, such as pulmonary artery pressure and pulmonary vascular resistance between the patients with open fenestration and the ones with closed fenestration. Patients with an open fenestration had significantly more late deaths (P < .001), Fontan failure (P = .021), and Fontan complications (P = .011) compared with those with a closed fenestration. Multivariable Cox regression revealed open fenestration (P < .001) and indeterminate ventricular morphology (P = .002) as risk factors for death/Fontan failure, and ventricular dysfunction (P = .014) and open fenestration (P = .009) as risk factors for Fontan complications. CONCLUSIONS: Persistent fenestration was a marker for physiologic intolerance as noted by increased rates of mortality and a higher incidence of Fontan failure/complications. The specificity of pre-Fontan physiologic data for fenestration status may not have the fidelity needed for long-term care and thus, the consequences of decision making regarding fenestration status may not be determined until well after the operation.

DOI： 10.1016/j.jtcvs.2014.06.062

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BACKGROUND: Extended end-to-end anastomosis (EEEA) through a left thoracotomy for coarctation of the aorta (CoA) and tubular hypoplasia of the aortic arch (THAA) leaves an unaugmented hypoplastic proximal aortic arch (PAA) segment, which may increase late reintervention for PAA obstruction. We sought to assess PAA growth and reintervention for PAA obstruction after EEEA. METHODS: Preoperative and follow-up echocardiographic images of 140 patients who underwent EEEA for CoA from 2005 to 2012 were reviewed. Patients were divided into two groups on the basis of preoperative PAA z-scores: THAA group, z-score less than -3; non-THAA group, z-score greater than or equal to -3. RESULTS: Eighty (57%) patients were identified as having THAA. There were three surgical reinterventions (PAA in 2 patients and distal aortic arch in 1 patient) and nine catheter reinterventions (all related to anastomotic stenosis) during a median follow-up period of 18 months. Both patients who required PAA reintervention had preoperative PAA z-scores below -8. Freedom from reintervention at 3 years was comparable between the groups (THAA group, 90.0% vs non-THAA group, 87.9%, p = 0.483). Follow-up echocardiography revealed PAA catch-up growth in the THAA group (z-score, preoperative -4.63 vs follow-up -1.17, p < 0.001); however, there was a nonsignificant trend toward smaller PAA in the THAA group (z-score: THAA, -1.17 vs non-THAA, -0.55, p = 0.057). All but 2 patients with preoperative PAA z-scores above -6 did not have any PAA obstruction. CONCLUSIONS: The hypoplastic PAA segment in patients with CoA/THAA grew significantly after EEEA but remained smaller than in those without THAA. Our data support that CoA and PAA with z-scores as small as -6 can be repaired through a thoracotomy approach with a low risk of reintervention.

DOI： 10.1016/j.athoracsur.2014.05.042

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BACKGROUND: The creation or enlargement of an atrial septal defect (ASD) in partial anomalous pulmonary venous drainage (PAPVD) repair may pose a risk of postoperative pulmonary vein stenosis (PVS), superior vena cava stenosis (SVCS), and atrial rhythm disturbances. METHODS: 155 children who underwent repair of right PAPVD between 1990 and 2010 were reviewed. PVS and SVCS were defined by mean gradients on echocardiography: mild=3 to 5 mm Hg; severe=6 mm Hg or higher. Postoperative cardiac rhythms were categorized as sinus, transient nonsinus, and persistent nonsinus rhythms. Outcomes were compared between patients who underwent the creation or superior enlargement of an ASD (group A) and those who did not (group B). RESULTS: There was no early or late death. Freedom from any PVS at 15 years after operation was lower in group A than in group B (76.1% vs 96.5%, p=0.002), and no differences were found in freedom from severe PVS (p=0.103), any SVCS (p=0.419), or severe SVCS (p=0.373). Group A patients had more PVS-related reoperations (p=0.022). Nineteen patients had nonsinus rhythm, and 4 patients experienced first-degree atrioventricular block, but no significant difference was found between the groups. Cox regression revealed the creation or superior enlargement of an ASD as a predictor for postoperative PVS (p=0.032). A case-match analysis confirmed a higher risk of PVS in patients with the creation or superior enlargement of an ASD (p=0.018). CONCLUSIONS: Late outcomes after repair of PAPVD are excellent. The subgroup that requires creation or superior enlargement of an ASD in repair of a right PAPVD is at a higher risk of late PVS and a subsequent increase in PVS-related reoperation. The presence of restrictive ASD did not increase SVCS, sinus node, or atrial conduction dysfunction.

DOI： 10.1016/j.athoracsur.2014.01.051

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OBJECTIVES: Transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO) with or without ventricular septal defect have multiple surgical treatment options. We sought to identify pre- and intraoperative factors that determine the timing of repair, procedure type and subsequent LVOT outcome. METHODS: Twenty-eight (8.2% of all TGA) patients with TGA with LVOTO (double outlet ventricle, n = 5, TGA/intact septum, n = 1) between 2000 and 2012 were reviewed. Anatomical factors were identified by prerepair echocardiography. LVOTO complexity was characterized by the degree of obstruction (0 = none, 0.33 = mild, 0.66 moderate and 1 = severe) at various levels: pulmonary valve (PV) dysplasia/hypoplasia, posterior deviation of the infundibular septum, fibromuscular ridge, tissue tag and abnormal chordal attachment. Summation of the obstruction score, at each level, yielded the LVOT complexity score. The descriptive analysis of intraoperative decision-making at late repair was performed. OPERATIONS: early arterial switch operation (ASO) + LVOT resection (n = 9, 32%), late ASO + LVOT resection (n = 3, 10%), Nikaidoh (n = 8, 29%), Rastelli (n = 6, 21%), single-ventricle palliation (n = 2, 7%). The primary LVOT obstruction mechanism was posterior deviation of the infundibular septum (n = 16, 57%) and PV dysplasia (n = 6, 21%). The early ASO group had a lower PV complexity score (0.42 ± 0.22 vs 0.96 ± 0.55, P = 0.007), tissue tag score (0.03 ± 0.15 vs 0.26 ± 0.34, P = 0.018) and LVOT complexity score (2.11 ± 0.86 vs 3.2 ± 0.96, P = 0.006). The LVOT complexity score in the Nikaidoh group was higher than in the late ASO group (P = 0.019). Of 16 candidates for the Nikaidoh procedure, 6 patients underwent a Rastelli operation due to coronary artery patterns (single coronary, n = 3, 1RL-2Cx, n = 2 or an abnormal left anterior descending coronary artery course, n = 1). Two patients underwent single-ventricle palliation due to the interference of essential chordae. All patients survived the operation. The 3-year survival was 96%. One patient who underwent late ASO required re-LVOT resection. CONCLUSIONS: A newly developed scoring system, the LVOT complexity score, helped to quantify the LVOT complexity and was correlated with our choice of the surgical procedure of TGA with LVOTO. The current strategy achieved reasonable survival and LVOT outcome with three quarters of the patients having an anatomically aligned LVOT. The coronary anatomy pattern was the primary determinant in the decision-making between the Nikaidoh procedure and the Rastelli operation.

DOI： 10.1093/ejcts/ezt283

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BACKGROUND: Reconstruction of anomalous left pulmonary veins (ALPV) requires an anastomosis at a nonanatomic position, posing the potential risk of pulmonary vein obstruction (PVO). METHODS: The 514 patients who were diagnosed with a pulmonary vein abnormality from 1990 to 2010 were reviewed. Thirty-eight patients (7.4%; median age, 1.4 years; interquartile range, 0.1 to 5.7 years) were identified. ALPV was diagnosed as an isolated anomaly in 23 (61%) or as part of mixed total anomalous pulmonary venous drainage in 15 (39%). Patients were divided into 3 groups (group 1: partial ALPV, treated; group 2: total ALPV, treated; or group 3: partial ALPV, untreated). Freedom from poor clinical (death/reoperation) and functional (any PVO, mean pressure gradient > 3 mm Hg) outcomes were analyzed. RESULTS: Repair in 30 ALPV patients (79%) was performed with direct anastomosis to the left atrium (n = 26 [68%]) or by sutureless repair (n = 4 [11%]). Two deaths occurred in group 2 (heart failure and PVO). The 4 reoperations in group 2 were prompted by PVO and occurred within 6 months of the initial repair. There was a nonsignificant trend of lower freedom from poor outcomes in group 2 (74.6%) vs group 1 (100%) at 10 years (p = 0.105). There was no difference in the incidence of any left PVO among the groups (p = 0.381). Severe left PVO did not develop in group 3 (n = 8 [21%]). CONCLUSIONS: Total ALPV carries a high risk of early PVO. Thus, the optimal surgical approach remains elusive. Untreated partial ALPV remained unobstructed during midterm follow-up. Therefore, surgical treatment may not be necessary in patients with partial ALPV.

DOI： 10.1016/j.athoracsur.2013.05.101

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Language：English   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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Language：English   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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Authorship：Lead author   Language：English   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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BACKGROUND: The use of a fenestration in the Fontan pathway remains controversial, partly because its hemodynamic effects and clinical consequences are insufficiently understood. The objective of this study was to quantify the magnitude of fenestration flow and to characterize its hemodynamic consequences after an intermediate interval after surgery. METHODS: Twenty three patients with a fenestrated extracardiac conduit prospectively underwent investigation by cardiac magnetic resonance (CMR), echocardiography, and invasive manometry under the same general anesthetic 12 ± 4 months after Fontan surgery. Fenestration flow was determined using phase contrast CMR by subtracting flow in the Fontan pathway above the fenestration from Fontan flow below the fenestration. RESULTS: Fenestration flow constituted a mean of 31 ± 12% (range 8-50%) of ventricular preload. It was associated with a lower Qp/Qs (r = -0.64, p=0.001) and oxygen saturation (r = -0.74, p<0.0001). Fenestration flow volume was correlated with pulmonary vascular resistance (r = 0.45, p = 0.04) and markers of ventricular diastolic function (early diastolic strain rate r = 0.57, p = 0.008 and ventricular untwist rate r = 0.54, p = 0.02). In 14 patients (61%) all of the net inferior vena cava flow and part of the superior vena cava flow were diverted into the systemic atrium and did not reach the lungs. CONCLUSIONS: Fenestration flow can be measured accurately with CMR. In two-thirds of the patients not only all of the inferior vena cava flow, but also some of the superior vena cava flow is diverted through the fenestration. Fenestration flow is driven by a balance between pulmonary vascular resistance and early diastolic ventricular function.

DOI： 10.1016/j.ijcard.2012.10.008

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BACKGROUND: All 3 palliation strategies, Norwood, Sano, and Hybrid, currently used for hypoplastic left heart syndrome pose a risk of myocardial injury at different times and through different mechanisms. We sought to compare these strategies to understand longitudinal differences in interstage ventricular dysfunction and their subsequent impact on transplant-free survival and atrioventricular valve regurgitation (AVVR) as well as the relationship between adverse events and ventricular function. METHODS AND RESULTS: Serial echocardiographic reports and clinical data were reviewed for 138 children with hypoplastic left heart syndrome who underwent stage I surgical palliation (Sano: 11; Norwood: 73; Hybrid: 54) between 2004 and 2011. Stage II palliation was achieved in 92 (67%) patients (Sano: 7; Norwood: 51; Hybrid: 34). Interstage transplant-free survival, ventricular dysfunction, and AVVR were equivalent among palliation strategies. Patients with preserved ventricular function had a higher rate of transplant-free survival and freedom from AVVR, regardless of palliation strategy. Patients who had cardiac arrest, cardiopulmonary resuscitation, or extracorporeal membrane oxygenation (adverse events) experienced more transient and persistent ventricular dysfunction compared to those without adverse events. Surgical palliation strategies were not identified as risk factors for ventricular dysfunction or AVVR. CONCLUSIONS: Surgical palliation strategy does not affect mortality, interstage ventricular function, or interstage AVVR in children with hypoplastic left heart syndrome. Therefore, the different timing and mechanisms of myocardial injury among palliation strategies do not affect outcomes. Ventricular dysfunction adversely affects transplant-free survival and atrioventricular valve function. Adverse events are associated with the development of ventricular dysfunction. To improve outcomes, interstage treatment should focus on the preservation of ventricular function.

DOI： 10.1161/CIRCULATIONAHA.112.000380

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BACKGROUND: There are a wide variety of reported techniques with few comparative trials and no current data available by which surgeons can compare their myopreservation strategies across the specialty. We therefore surveyed congenital heart surgeons to develop a profile of current practice. METHODS: One hundred twenty-two members of the Congenital Heart Surgeons' Society were surveyed, and 56 responses were analyzed. The survey focused on cardioplegia formulations, dosage and administration, and perfusion strategies for four age groups: neonates, infants, children, and adolescents. All percentages are expressed as percentage of the entire reporting cohort (n=56). RESULTS: Eighty-six percent of surgeons use blood-based cardioplegia versus crystalloid cardioplegia. Microplegia is used in 5%. Blood-based cardioplegia additives include del Nido (38%), customized solutions (32%), St. Thomas, Plegisol, or Baxter (11%), and microplegia (5%). Crystalloid cardioplegia types are Custodiol (7%), St. Thomas, Plegisol, or Baxter (5%), and customized solutions (2%). Cold (<10°C) cardioplegia is most common (93%), and "hot shots" are used in 21%. Moderate (26° to 30°C) hypothermic cardiopulmonary bypass is more common in neonates and infants compared with older children and adolescents. Antegrade administration is most common (89%). Longer intervals between cardioplegia doses were associated with surgeons using del Nido and Custodiol solutions, and these solutions were commonly administered with a single dose regardless of aortic cross-clamp time. CONCLUSIONS: Myocardial protection techniques still remained highly variable among congenital heart surgeons. This survey demonstrates that there is a perception that del Nido and Custodiol solutions can offer appropriate myocardial protection for longer intervals with decreased repeat dosing. An observational study correlating markers of postoperative myocardial performance with myocardial preservation strategies should be considered.

DOI： 10.1016/j.athoracsur.2013.05.052

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BACKGROUND: The frequency and impact of medical errors during staged palliation are unknown. METHODS: All patients with hypoplastic left heart syndrome and physiologic equivalents (N = 191) who underwent staged palliation (2001-2011) were studied. Stage 1, interstage, and stage 2 were reviewed to identify diagnostic, technical, judgment, and management errors. The impact of errors on transplant-free survival was examined by parametric competing risks and risk-adjusted regressions using bootstrapping. RESULTS: Stage 1 (N = 191) errors (n = 111, 58%) were common and predominantly intraoperative (n = 84, 44%) or postoperative (n = 43, 23%). Postoperative errors were determinants of death/transplant (hazard ratio, 1.7; P = .01), whereas technical errors (n = 65, 34%) were not, but they delayed recovery and discharge (extra 24 days approximately, P = .0024). Postoperative stage 1 errors led to decrements in total strategy success of approximately 30% (78% vs 48%, P = .004). Stage 2 (N = 134) errors (n = 66, 49%) were common. Intraoperative errors were the most prevalent (n = 61, 46%) but did not compromise survival. Postoperative errors (n = 11, 8%) were determinants of death/transplant (hazard ratio, 2.4; P < .0001). Interstage errors (n = 21, 16%) led to twice the intensive care unit stay (16 vs 7 days, P < .0001) and hospital stay (30 vs 17 days, P < .02) after stage 2. Overall, a child presenting with ideal morphology and managed with no postoperative errors at stage 1 or 2 would have a predicted late survival in excess of 80%. CONCLUSIONS: Technical errors are common and delay recovery. Their effects on survival are mitigated. Intraoperative judgment errors are associated with strategy failure in a univariate model and lead to increased postoperative errors in a multivariate model. Postoperative errors are independently associated with a decrease in univentricular strategy survival.

DOI： 10.1016/j.jtcvs.2012.12.065

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OBJECTIVES: We sought to elucidate late functional outcomes of the right ventricular outflow tract and atrioventricular valves after repair of tetralogy of Fallot with atrioventricular septal defect. METHODS: From 1990 to 2010, the data from 41 patients who underwent repair were retrospectively reviewed. The median age at repair was 22.4 months (interquartile range, 10.8-41.6 months). Of the 41 patients, 13 (32%) had received previous palliations. The preoperative anatomic differences, physiologic differences, and long-term functional outcomes were determined using a double case-matched control with isolated tetralogy of Fallot and isolated atrioventricular septal defect. RESULTS: The right ventricular outflow tract was reconstructed, with the pulmonary valve preserved in 23 patients (56%). There were 3 early deaths and no late deaths. Survival was 92.1% at 15 years. During a median follow-up period of 5.9 years (interquartile range, 0.14-13.7 years), 29 reinterventions were performed in 13 (32%) patients. Freedom from all reintervention at 15 years was 52.8%. Of the 29 procedures, 12 (41%) were related to the right ventricular outflow tract. Freedom from right ventricular outflow tract-related reintervention was greater in patients who had their pulmonary valve preserved (95% vs 70% at 10 years; P = .046). Reinterventions were performed for the atrioventricular valve in 2 (5%) patients and subaortic stenosis in 1 (2%) patient. Freedom from right ventricular outflow tract-related reintervention (tetralogy of Fallot with atrioventricular septal defect, 88.6% vs tetralogy of Fallot alone, 83.9% at 5 years; P = .809) and atrioventricular valve/left ventricular outflow tract-related reintervention (tetralogy of Fallot with atrioventricular septal defect, 95.2% vs atrioventricular septal defect alone, 86.0% at 5 years; P = .332) were comparable between the tetralogy of Fallot with atrioventricular septal defect and matched control groups. CONCLUSIONS: Late survival and atrioventricular valve function after repair of tetralogy of Fallot with atrioventricular septal defect were excellent. Pulmonary valve preservation and avoidance of an artificial conduit were associated with greater freedom from right ventricular outflow tract reintervention. In the current era, the surgically modified history of tetralogy of Fallot with atrioventricular septal defect is not significantly different from that of isolated tetralogy of Fallot or isolated atrioventricular septal defect.

DOI： 10.1016/j.jtcvs.2013.01.007

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Technological development has had a tremendous impact on the management of patients who require extracorporeal membrane oxygenation (ECMO). Team development and education are a vital component of a successful extracorporeal life support (ECLS) Program to reduce complications and subsequently improve clinical outcomes. We sought to review the evolution in technology, importance of team development and training, and report our experience at The Hospital for Sick Children, Toronto. There were a total of 576 ECMO runs in 534 patients (42 repeat ECMO runs) between January 1988 and June 2012. The use of ECMO for cardiac disease has increased in the last decade due to an expanded indication for ECMO in patients with single-ventricle physiology. Cardiac ECMO still remains a challenge in terms of survival (177/392, 45%). Although development of an ECLS program and team education facilitated extracorporeal cardiopulmonary resuscitation, clinical outcomes were not satisfactory (survival, 33%). The most common complications were hemorrhagic (13.8%), followed by renal (10.6%) and pulmonary dysfunction (6.9%). Advances in technology made management during ECMO safer, and the mechanical complications related to the ECMO system were 6.1%, including circuit changes due to thrombus formation, cannula repositioning, or optimization of size.

DOI： 10.1111/aor.12032

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From 2005 to 2011, 23 of 178 (12.9%) patients with venoarterial (VA) extracorporeal membrane oxygenation (ECMO) had left atrial (LA) decompression to help improve left ventricular (LV) function, LA/LV dilatation, and/or lung edema. LA decompression was achieved with LA cannulation (n = 16), surgically created adjustable atrial septal defect (n = 3), or balloon atrial septostomy (n = 4). Sixteen (70%) patients had LA decompression at the time of ECMO initiation and all had LA decompression within 12 hours of ECMO initiation. ECMO duration was 5.9 ± 4.5 days and 16 (70%) patients were successfully decannulated. Subsequent intensive care unit and hospital survival was achieved in 13 (57%) and 12 (52%) patients, respectively. Earlier timing of LA decompression appeared to be associated with a high probability of weaning from ECMO and reasonable LV functional recovery.

DOI： 10.1111/j.1525-1594.2012.01534.x

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BACKGROUND: The durability of atrioventricular valve (AVV) repair and risk factors for recurrent AVV regurgitation (AVVR) and reintervention in single-ventricle patients are not well defined. METHODS: Among 66 single-ventricle patients who underwent AVV repair between 1998 and 2011, 58 hospital survivors (88%) were retrospectively reviewed. Freedom from recurrent AVVR and reintervention were analyzed with Kaplan-Meier analysis. Predictors for recurrent AVVR, ventricular dysfunction, and reintervention were analyzed using regression analysis. RESULTS: Significant (more than mild+) AVVR developed in 47 patients (81%) during mean follow-up of 37 months (range, 0.2 to 103 months). Freedom from significant AVVR was 23.8% at 1 year and 16.9% at 5 years. Reintervention was performed in 12 patients (26%) at a mean of 24 months (range, 2 to 64 months) after the initial repair. Freedom from reintervention was 92.3% at 1 year and 75.3% at 5 years. There were 11 late deaths (19%). Predictors for recurrent AVVR included repair at stage II (p=0.020) and cardiopulmonary bypass time (p=0.014). Predictors for reintervention included valvuloplasty as a repair technique (p=0.013), cardiopulmonary bypass time (p=0.002), aortic cross-clamp time (p=0.003), and significant residual intraoperative AVVR (p=0.012). Intraoperative ventricular dysfunction (p<0.001), aortic cross-clamp time (p=0.005), and cleft as the mechanism of regurgitation (p=0.023) predicted postrepair ventricular dysfunction. CONCLUSIONS: Although significant AVVR developed in most patients within 1 year of repair, the need for repeat valve repair is relatively low if ventricular function is preserved. Ventricular function after repair did not predict late survival but was related to the longevity of AVV competence and subsequent risk for reintervention.

DOI： 10.1016/j.athoracsur.2012.04.048

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OBJECTIVE: Aortopulmonary collaterals are a frequent phenomenon in patients after bidirectional cavopulmonary connection. The aortopulmonary collateral flow volume can be quantified using cardiac magnetic resonance imaging. However, the significance of aortopulmonary collateral flow for the postoperative outcome after total cavopulmonary connection is unclear and was sought to be determined. METHODS: The data from 33 patients were prospectively studied with cardiac magnetic resonance, echocardiography, and cardiac catheterization before the total cavopulmonary connection operation. The early postoperative outcomes after total cavopulmonary connection completion were recorded. RESULTS: Aortopulmonary collateral flow was 1.59 L/min/m(2) ± 0.65 L/min/m(2) (range, 0.54 L/min/m(2)-3.34 L/min/m(2)), constituting 43% ± 13% (range, 12-87%) of pulmonary blood flow and 35% ± 12% (range, 11-62%) of the cardiac index, resulting in a pulmonary blood flow/systemic blood flow ratio of 1.06 ± 0.17 (range, 0.79-1.55). The aortopulmonary collateral flow correlated with pulmonary blood flow/systemic blood flow ratio (r = 0.69, P < .0001), oxygen saturation (r = 0.42, P = .018), and cardiac index (r = 0.53, P = .002). Of the 36 patients, 24 underwent fenestrated total cavopulmonary connection during the study period. The aortopulmonary collateral flow, relative to the cardiac index, correlated with the duration of hospital stay (r = 0.48, P = .02) and pleural drainage (r = 0.45, P = .03). Patients whose pleural drainage lasted 1 week or less had less aortopulmonary collateral flow before the Fontan operation than those with a longer period until chest tube removal (1.23 L/min/m(2) ± 0.38 L/min/m(2) vs 1.73 L/min/m(2) ± 0.76 L/min/m(2); P = .03). Compared with a contemporary group of total cavopulmonary connection patients with fenestration in their extracardiac conduit who were studied prospectively, with a similar protocol, the bidirectional cavopulmonary connection had a greater amount of aortopulmonary collateral flow (1.59 L/min/m(2) ± 0.65 L/min/m(2) vs 1.30 L/min/m(2) ± 0.57 L/min/m(2), P = .04). CONCLUSIONS: Patients after bidirectional cavopulmonary connection routinely acquire a large amount of aortopulmonary collateral flow. The hemodynamic consequences of aortopulmonary collateral flow translate into adverse outcomes early after total cavopulmonary connection completion.

DOI： 10.1016/j.jtcvs.2012.03.032

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BACKGROUND: This study evaluated the effect of a new measurement-superior vena cava (SVC) flow-and anatomic factors on postoperative arterial oxygen saturation and clinical outcome in patients who underwent bidirectional cavopulmonary shunt (BCPS). METHODS: We reviewed 19 patients who underwent a BCPS between January 2009 and May 2011 who also had SVC blood flow measurements. Median age was 6 months (range, 2 to 29 months). Body weight was 7.0 ± 1.7 kg. Bilateral SVCs were present in 4. SVC flow was measured at the time of BCPS by a Transonic flow probe (Transonic Systems Inc, Ithaca, NY). SVC flow, preoperative hemodynamics, pulmonary artery size, and clinical outcome were analyzed to determine risk for morbidity and death. RESULTS: Mean absolute and indexed pre-BCPS SVC flow was 0.65 ± 0.23 L/min and 1.63 ± 0.55 L/min/m(2) or 91.1 ± 30.8 mL/kg/min, respectively. In all but 1 patient, the SVC flow was increased after BCPS from 1.63 ± 0.55 to 1.99 ± 0.57 L/min/m(2) (p = 0.005). There was a significant positive correlation between pre-BCPS and post-BCPS SVC flow (r = 0.627, p = 0.029). Pulmonary artery size correlated with post-BCPS SVC flow (r = 0.560, p = 0.016). Two patients with preoperative SVC flow of below 1.0 L/min/m(2) died or required BCPS takedown. SVC size did not correlate with BCPS flow (r = 0.231, p = 0.356). Univariate analysis indicated pre-BCPS pulmonary artery pressure was a risk factor for low arterial oxygen saturation (≤ 75%) immediately after BCPS (p = 0.042) and at discharge (p = 0.030). CONCLUSIONS: A new indicator-low SVC flow, may be a marker for BCPS failure or death, suggesting that the SVC flow vs size is more important in predicting successful BCPS.

DOI： 10.1016/j.athoracsur.2012.05.043

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Authorship：Lead author   Language：English   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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BACKGROUND: Hybrid and Norwood strategies differ substantially in terms of stage II palliative procedures. We sought to compare these strategies with an emphasis on survival and reintervention after stage II and subsequent Fontan completion. METHODS AND RESULTS: Of 110 neonates with functionally single-ventricle physiology who underwent stage I palliation between 2004 and 2010, 75 (69%) infants (Norwood, n=43; hybrid, n=32) who subsequently underwent stage II palliation were studied. Survival and reintervention rates after stage II palliation, anatomic and physiologic variables at pre-Fontan assessment, and Fontan outcomes were compared between the groups. Predictors for reintervention were analyzed. Freedom from death/transplant after stage II palliation was equivalent between the groups (Norwood, 80.4% versus hybrid, 85.6% at 3 years, P=0.66). Hybrid patients had a higher pulmonary artery (PA) reintervention rate (P=0.003) and lower Nakata index at pre-Fontan evaluation (P=0.015). Aortic arch and atrioventricular valve reinterventions were not different between the groups. Ventricular end-diastolic pressure, mean PA pressure, and ventricular function were equivalent at pre-Fontan assessment. There were no deaths after Fontan completion in either group (Norwood, n=25, hybrid, n=14). CONCLUSIONS: Survival after stage II palliation and subsequent Fontan completion is equivalent between the groups. The hybrid group had a higher PA reintervention rate and smaller PA size. Both strategies achieved adequate physiology for Fontan completion. Evolution of the hybrid strategy requires refinement to provide optimal PA growth.

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：IEICE-INST ELECTRONICS INFORMATION COMMUNICATIONS ENG  

In this paper, a novel charge coupled device matched filter (CCD-MF) for Electrical code division multiplexing (ECDM) decoder is proposed and experimentally demonstrated. Simulation results clarify the influence of low charge transfer efficiency (CTE) and the validity of a parallel CCD-MF we proposed. A 15-channel ECDM system using a 2 Gchip/s, 2-parallel CCD-MF is experimentally demonstrated.

DOI： 10.1587/transcom.E95.B.2568

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We report 4 cases of left main coronary artery (LMCA) compression after remote repair of conotruncal lesions and their successful surgical management.

DOI： 10.1016/j.athoracsur.2011.12.048

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Linear motors have many applications because of their unique characteristic which are different from rotary machines. In particular, Linear Induction Motors (LIMs) have a simple and strong structure, a flexible mechanism, and so on. The most popular application of LIMs is in transportation systems. It is important to investigate the dynamic characteristics of LIMs in order for them to be successfully applied in transportation systems. However, it is difficult to measure the dynamic characteristics of LIMs. Long enough span or large enough diameter of a cylindrical drum is necessary for the secondary side in the ordinary measurement system.In this paper, the use of a disc-shaped secondary side to measure the dynamic characteristics of LIMs are proposed. The rotating dynamic characteristics of disc-shaped LIMs are converted into linear motion.

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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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We developed a new cardiopulmonary bypass (CPB) method to minimize myocardial damage during aortic arch reconstruction. In this method, coronary flow and heartbeat were stabilized by maintaining the aortic root pressure with an adjusted preload of the ventricle during aortic cross-clamping. This study was performed to determine the appropriate root pressure to maintain the heartbeat without causing deterioration of ventricular function. Study 1. Under partial CPB, the ascending aorta was cross-clamped in 6 pigs (group 1). Experimental data at various systolic aortic root pressures was analysed to determine the appropriate root pressure. Study 2. In group 2 (control, n＝6), the aorta was not clamped, while in group 3 (n＝6), the aorta was cross-clamped for 60 min and the systolic aortic root pressure was maintained at the pressure determined in study 1. Study 1. The diastolic coronary flow was stabilized at values comparable to that before initiation of CPB (6.6±1.4 ml/beat) when the systolic aortic root pressure was above 80 mmHg. Intracardiac pressure and the myocardial oxygen consumption (MvO2) seemed to be acceptable when the systolic aortic root pressure was below 100 mmHg. Therefore, 90 mmHg was selected for study 2. Study 2. Perioperative cardiac function did not differ between the groups. We concluded that 90 mmHg was the systolic aortic root pressure appropriate for this method.

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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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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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DOI： 10.1016/j.jtcvs.2009.07.021

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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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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：IEEE-INST ELECTRICAL ELECTRONICS ENGINEERS INC  

In this letter, 1.25 Gb/s x 8-channels simultaneous multiplexing using 8-chip electrical codes is experimentally demonstrated for the downlink of a 10-Gb/s electrically code-division-multiplexing passive optical network (ECDM-PON) system. For electrical encoding and decoding, 10-Gb/s multilevel signal generation and 10 Gchip/s correlator as a decoder are utilized. We demonstrate 10-Gb/s ECDM error-free optical transmission over 20 km of single-mode fiber.

DOI： 10.1109/LPT.2010.2047010

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BACKGROUND: To determine diagnosis-based differences in the response of global ventricular performance to modified ultrafiltration (MUF) using transesophageal echocardiography during congenital heart surgery. METHODS AND RESULTS: The study included 38 children with atrial septal defect (n=10), ventricular septal defect (VSD) (n=8), tetralogy of Fallot (TOF) (n=9), or a single ventricle (n=11). Arteriovenous MUF was performed for 10-15 min after cardiopulmonary bypass (CPB). The myocardial performance index (MPI) of the systemic ventricles and the % change in MPI before and after MUF were assessed. Impairment of MPI was noted at termination of CPB compared with baseline values in the VSD and TOF groups (P<0.05). MUF resulted in an improvement in MPI in all groups (P<0.01). There was a weak correlation between aortic cross-clamping or CPB time, and the degree of improvement in MPI (r= 0.385, P=0.019; r= 0.348, P=0.037, respectively). MUF improved fractional shortening in all groups (P<0.05) and reversed abnormal relaxation in the VSD and TOF groups. CONCLUSIONS: Modified ultrafiltration ameliorated MPI in all groups, indicating improved systemic ventricular function with MUF. The MPI recovery rate differed among the groups. MUF may be particularly useful for restoring the global ventricular performance of patients undergoing longer CPB and may have minimal advantages for simple open-heart surgery. (Circ J 2010; 74: 86 - 92).

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First time, we have experimentally demonstrated 1.25-Gbps/subscriber 10-Gchip/s ECDM-PON upstream transmission over 20-km SSMF using WDM light sources. Additionally, optical beating induced performance degradation was investigated by varying WDM channel spacing from 100-GHz to 10-GHz.

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First time 8-channels electrically code division multiplexing technique for optical access networks is demonstrated at a chip-rate of 10 Gchip/s. The technique enables error-free transmission of 1.25 Gbit/s x 8-channels over 20 km single-mode-fiber. (C) 2010 Optical Society of America

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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 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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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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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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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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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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BACKGROUND: This study analyzed the clinical application of modified low-flow ultrafiltration (MUF) to minimize cardiopulmonary bypass (CPB)-related adverse effects in patients undergoing living-donor lobar lung transplantation (LDLLT). METHOD: The study enrolled 33 consecutive patients who underwent LDLLT from 1999 to 2004: 8 patients underwent conventional CPB without MUF (control group), and 15 underwent arteriovenous MUF (MUF-treated group). Hemodynamics, graft function, blood loss and blood transfusion requirements, and clinical outcomes were analyzed. RESULTS: There was a significant increase in systolic blood pressure and a decrease in pulmonary to systemic pressure ratio in the MUF-treated group (p < 0.05). No hemodynamic changes occurred in the control group. MUF resulted in significant improvements in arterial oxygen tension/fraction of inspired oxygen ratio (PaO(2)/FiO(2;) 411 +/- 107 vs 272 +/- 107 mm Hg, p < 0.05) and the alveolar-arterial oxygen difference (a-aDO(2); 158 +/- 84 vs 315 +/- 127 mm Hg, p < 0.05) at 15 minutes after CPB. There were no differences in PaO(2)/FiO(2) and A-aDO(2) between the groups beyond 6 hours post-operatively. Post-operative blood loss and blood transfusion requirements were lower in the MUF-treated group than in the control group (p < 0.05). There were no differences in survival, duration of ventilation, intensive care unit stay, and hospital stay between the groups. CONCLUSIONS: The low-flow MUF brought improved hemodynamics and gas exchange capacity of transplanted grafts and lowered post-operative blood loss and blood transfusion requirement. This strategy may minimize CPB-related adverse effects in patients undergoing LDLLT.

DOI： 10.1016/j.healun.2009.01.004

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BACKGROUND: Circulatory load during hypoxia is unavoidable in donation after cardiac death (DCD) hearts, but it causes severe myocardial damage. The impact of circulatory load on donor heart function has not been investigated. The purpose of this study was to evaluate its effect on post-transplant functional recovery of DCD hearts. METHODS: Twelve donor pigs (20 kg) were used. Cardiac arrest was induced by asphyxiation (turning off the ventilator) in the load group (n = 6) and by exsanguination (dividing the vena cava) in the unload group (n = 6). Left ventricle end-diastolic volume (LDEDV) and end-systolic pressure (LVESP) were monitored until cardiac arrest. Orthotopic transplantation was performed after 30-minute warm ischemia following cardiac arrest. After weaning from cardiopulmonary bypass, left ventricular end-diastolic pressure-volume ratio (LV Emax) and creatine kinase (CK-MB) were measured while on 0.1 microg/kg/min epinephrine. RESULTS: During the agonal period, the maximum LVEDV and LVESP in the load group were 132 +/- 1% of baseline at 10 minutes and 148 +/- 16% of baseline at 4 minutes, respectively. Recovery rates of post-transplant cardiac function in the load group were worse than in the unload group (LV Emax: 64 +/- 8 vs 84 +/- 5%, p < 0.05). Levels of post-transplant CK-MB in the load group were higher than in the unload group (639 +/- 119 vs 308 +/- 70 IU/liter, p < 0.05). CONCLUSIONS: Cardiac arrest with circulatory load by asphyxiation caused more myocardial damage than unloaded arrest. This difference between the modes of death should be considered when evaluating the DCD hearts for clinical application.

DOI： 10.1016/j.healun.2008.12.002

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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：English   Publisher：CHURCHILL LIVINGSTONE INC MEDICAL PUBLISHERS  

DOI： 10.1016/j.cardfail.2008.07.093

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Language：English   Publisher：ELSEVIER IRELAND LTD  

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Language：English   Publisher：ELSEVIER IRELAND LTD  

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BACKGROUND: The present study analyzes a 15-year experience of repairing persistent truncus arteriosus (PTA) with a consistent policy of right ventricular outflow tract (RVOT) reconstruction (ie, direct anastomosis). METHODS AND RESULTS: This retrospective study included 13 consecutive patients with PTA (8 type I PTA, 5 type II) who underwent primary repair from September 1992 to December 2006. Median age and body weight at surgery were 21 days and 2.9 kg, respectively. All but 1 patient underwent RVOT reconstruction by direct anastomosis with a monocusp patch. There were 2 operative deaths (12%). No patient had a pulmonary hypertensive crisis. The median duration of ventilation was 5 days. Another patient died from cardiogenic shock resulting from late cardiac tamponade 2 months after surgery. Four patients (40%) required balloon angioplasty and 5 (50%) required re-operation for branch pulmonary artery and/or conduit obstruction during the median follow-up period of 70 months (44-174 months). Freedom from all re-interventions and re-operation at 5 years was 50% (95% confidence limits, 19-81%) and 60% (95% confidence limits, 30-91%), respectively. CONCLUSIONS: Reasonable early and long-term results can be achieved with direct anastomosis. Further reduction of the re-intervention rate could be attained by refining the surgical techniques and catheter intervention strategies.

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

We have developed a miniaturized semiclosed cardiopulmonary bypass (CPB) circuit incorporating a centrifugal blood pump (TinyPump) with a volume of 5 ml. The current study was undertaken to evaluate the hemolytic performance of the TinyPump in comparison with the BioPump and to investigate the impact of different CPB circuit volumes on hemodilution, coagulation, and the inflammatory response. Twelve 1-week-old piglets (3.4 +/- 0.2 kg) were used. The circuit comprised a centrifugal pump, a membrane oxygenator, and a cardiotomy reservoir. Cardiopulmonary bypass was conducted with mild hypothermia at 150 ml/kg/min for 3 hours. Transfusion was not performed. Priming volume was 68 ml for the circuit with the TinyPump and 111 nil for the circuit with the BioPump. Although the TinyPump required higher speed, plasma free hemoglobin levels after CPB were not different between the groups. After CPB, the TinyPump group had a significantly higher hematocrit (27% +/- 3% vs. 23% +/- 3%) and lower platelet reduction rate, lower thrombin-antithrombin complex levels, and lower interleukin-6 levels. Better lung compliance with less water content was observed in the TinyPump group. The TinyPump maintained CPB with acceptable hemolysis and lower inflammatory responses. This miniaturized CPB circuit may make transfusion-free open heart surgery feasible in neonates and would help to prevent postoperative organ dysfunction.

DOI： 10.1097/MAT.0b013e3181590719

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

OBJECTIVE: Oxygen-derived free radicals are responsible in part for reperfusion injury in globally ischemic myocardium. In this study, the efficacy for resuscitation of nonbeating donor hearts of MCI-186, a free-radical scavenger and antioxidant, was investigated in a pig transplantation model. METHODS: Cardiac arrest was induced by asphyxiation. After 30 minutes of global ischemia, the hearts were excised and immediately reperfused from the aortic root with normoxemic blood cardioplegia (PO2 100 mm Hg) for 20 minutes, followed by perfusion with hyperoxemic blood (PO2 300 mm Hg). MCI-186 (3 mg/kg) was administered into the aortic root for the first 30 minutes of reperfusion in the treated group (n = 6), and untreated hearts were used as a control group (n = 6). Transplantation was performed with the heart beating. RESULTS: Posttransplantation recovery of cardiac output, end-systolic pressure-volume ratio, and first derivative of pressure of the left ventricle in the treated group were significantly better than those in the control group. The coronary sinus-aortic root difference in malondialdehyde levels remained low throughout reperfusion in the treated group but abruptly increased after initiation of oxygenated blood perfusion in the control group. The MCI-186-treated hearts showed low degree of edema and well-preserved ultrastructure with normal-appearing organelles, whereas the untreated hearts had marked swelling of mitochondria and scant glycogen granules. CONCLUSION: MCI-186 exerts a cardioprotective action at least partly by inhibition of lipid peroxidation. Antioxidant therapy at the initial reperfusion is essential to successful resuscitation of nonbeating hearts by continuous myocardial perfusion.

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In this study, the performances of the TinyPump (priming volume 5 mL) system including the pediatric cannulae (Stöckert Pediatric Arterial Cannulae 2.6, 3.0, and 4.0 mm, Stöckert Instruments GmbH, Munich, Germany; Polystan 20-Fr Venous Catheter, MAQUET GmbH, Rastatt, Germany) and an oxygenator (Terumo Capiox RX05 Baby-RX, Terumo Cardiovascular Systems Co., Tokyo, Japan) were studied in vitro followed with preliminary ex vivo studies in 20-kg piglets. In vitro results revealed that the TinyPump system met the requirements for pump speed, pump flow, and pressure drop as extracorporeal circulatory support during open heart surgery and extracorporeal membrane oxygenation (ECMO) in pediatric patients. In 2-h ex vivo studies using 20-kg piglets where the blood contacting surface of the TinyPump was coated with a biocompatible phospholipid polymer, the plasma-free hemoglobin levels remained less than 5.0 mg/dL and no thrombus formation was observed inside the pump. The TinyPump system including the oxygenator and connecting circuits resulted in an overall priming volume of 68 mL, the smallest ever reported. The TinyPump can be a safe option for pediatric circulatory support during open heart surgery and ECMO without requiring blood transfusion.

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Left ventricular (LV) retraining followed by anatomical repair would be a superior alternative in patients with congenitally corrected transposition (ccTGA) having a deconditioned morphologically left ventricle (MLV); however, LV retraining in older children is a challenging task. A retraining process of the MLV in a teenage patient with ccTGA is reported here. Cardiac catheterization at 7 years of age revealed low pressure of the MLV (33/4 mm Hg) and a LV to right ventricular pressure ratio (LVp/RVp ratio) of 0.32. The first pulmonary artery banding (PAB) was performed at 10 years of age. Although the LVp/RVp ratio reached 0.68, there was no evidence of adequate LV hypertrophy. The second PAB was performed 2 years after the initial PAB, resulting in an increase in the LVp/RVp ratio to 0.93 and an adequate LV hypertrophy. The double switch procedure was successfully performed at 13 years of age. Although the ejection fraction of the MLV mildly decreased, the patient has been doing well during a follow-up period of 4 years. The MLV in the teenage patient with ccTGA was successfully trained using a retraining strategy and has sustained systemic circulation after anatomical repair.

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BACKGROUND: The purpose of this study was to investigate the discrepancy between intraoperative transesophageal and postoperative transthoracic echocardiography in assessing residual regurgitation in children undergoing valve repair. METHODS: Forty-two consecutive children (median age, 5.1 years) who underwent valve repair for valvar regurgitation from 2001 to 2004 were retrospectively analyzed. The patients were divided into two groups: atrioventricular valve group (n = 33) and aortic valve group (n = 9). Regurgitation grade, fractional shortening, and atrioventricular inflow velocity obtained by intraoperative transesophageal echocardiography were compared with those obtained by transthoracic echocardiography at discharge (median, 11 days) and at follow-up (median, 8 months). RESULTS: Intraoperative transesophageal echocardiography revealed specific residual lesions in 4 patients, leading to successful re-repair. Fractional shortening obtained by intraoperative transesophageal echocardiography was lower than that obtained by predischarge transthoracic echocardiography (p < 0.01). In the atrioventricular valve group, the regurgitation grade obtained by intraoperative transesophageal echocardiography was lower than that obtained by predischarge transthoracic echocardiography (0.7 +/- 0.8 versus 1.4 +/- 0.9; p < 0.01), and agreement between the two examinations was found in 12 patients (38%). Peak atrioventricular inflow velocity obtained by intraoperative transesophageal echocardiography was lower than that obtained by predischarge transthoracic echocardiography (p < 0.01). In the aortic valve group, there was no significant difference between the regurgitation grades in the two examinations (0.8 +/- 0.8 versus 1.1 +/- 0.9), and complete agreement in regurgitation grade was found in 5 (56%) of 9 patients. CONCLUSIONS: There were considerable discrepancies between the examinations in evaluation of residual atrioventricular valve regurgitation and potential atrioventricular valve stenosis: most of the residual regurgitations were underestimated by intraoperative transesophageal echocardiography. In contrast, reasonable agreement was found between the two examinations in evaluation of aortic valve regurgitation.

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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: Warm ischemia is a major cause of cardiac allograft failure in transplants from non-heart-beating donors. To minimize myocardial ischemia, we used a continuous myocardial perfusion technique for resuscitation of donor hearts. The purpose of the present study was to investigate an optimal duration of controlled initial reperfusion. METHODS: Cardiac arrest was induced by asphyxia in 18 donor pigs. The hearts were harvested 30 minutes after global warm ischemia. Continuous myocardial reperfusion was immediately commenced from the aortic root with blood cardioplegic solution (20 degrees C, 40 mm Hg) and then with oxygenated blood (20 degrees to 37 degrees C, 40 to 60 mm Hg). Animals were divided into three groups according to the duration of the initial reperfusion: group I = 5 minutes, group II = 20 minutes, and group III = 60 minutes. Orthotopic transplantation was performed while keeping the heart beating by continuous myocardial perfusion. Cardiac function was evaluated before anoxia and after transplantation. Lactate extractions were determined during reperfusion. Myocardial edema was assessed by heart weight and posterior wall thickness of the left ventricle. RESULTS: Recovery rates of cardiac function in group II hearts after transplantation were better than in groups I and III (cardiac output, 61% +/- 9% versus 41% +/- 5% versus 44% +/- 4%, respectively; p < 0.05; left ventricular end-systolic pressure-volume ratio, 64% +/- 8% versus 36% +/- 9% versus 42% +/- 6%, respectively; p < 0.05). Lactate extractions in groups II and III returned to 0 within 20 minutes of reperfusion. Myocardial edema after transplantation in group II hearts was less than in groups I and III. CONCLUSIONS: The best recovery was observed in the non-beating donor hearts resuscitated by continuous myocardial perfusion when the initial controlled reperfusion with lukewarm blood cardioplegic solution at 40 mm Hg lasted for 20 minutes.

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In the process of establishing a less invasive assessment strategy for coronary artery disease (CAD) in patients with infrarenal abdominal aortic aneurysm (AAA), the incidence of CAD and the surgical and mid-term outcomes were reviewed. From January 1994 through September 2001, 94 elective surgical repairs of AAA were carried out. Preoperative coronary angiography showed 43 patients (45.7%) had CAD: 29 (67.4%) were asymptomatic, 23 had single-vessel disease (1VD), 12 had 2VD and 8 had 3VD. Of the 43 patients with CAD, 19 (44.2%) underwent coronary interventional therapy before aortic surgery (11 percutaneous transluminal coronary angioplasty (PTCA), 8 coronary artery bypass grafting). Eight asymptomatic patients underwent coronary interventional therapy. None of the patients died of cardiac causes or experienced a postoperative cardiac event. During the follow-up period, 10 late deaths occurred: 7 patients with CAD, and 3 cerebrovascular or cardiac deaths. There was no statistical difference in the survival rate between the groups with and without CAD. Two patients with CAD underwent PTCA during the follow-up period. The findings confirm the need for a less invasive assessment strategy of CAD that does not overlook asymptomatic myocardial ischemia, because the incidence of CAD in patients with AAA is high.

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J-GLOBAL

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

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Language：Japanese   Publisher：(一社)日本移植学会  

J-GLOBAL

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

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Language：Japanese   Publisher：(一社)日本移植学会  

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J00083&link_issn=&doc_id=20221028010420&doc_link_id=%2Fcl8isoke%2F2022%2Fs057s1%2F183%2F0334-0334%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fcl8isoke%2F2022%2Fs057s1%2F183%2F0334-0334%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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

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Language：Japanese   Publisher：金原出版(株)  

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

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

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

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

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

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

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

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

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

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

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

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

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

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CiNii Books

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Other Link： http://search.jamas.or.jp/link/ui/2021220359

J-GLOBAL

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Language：Japanese   Publisher：(一社)日本小児外科学会  

症例は女児。在胎28週時に前縦隔に径14mmの嚢胞性病変を指摘された。その後、大きさや性状に変化は認めなかった。在胎38週1日、体重3,036gで出生した。CTやMRIにて胸腺右葉下極に嚢胞を含む内部不均一な腫瘤を認めたが、この腫瘤は胸腺左葉下極の内部均一な嚢胞状腫瘤と正中で接していた。胸腺右葉の奇形腫と、奇形腫が胸腺左葉内に穿破して嚢胞を形成したと考えられた。出生直後は無症状であったが、日齢18頃に啼泣時の経皮動脈血酸素飽和度の低下を認めるようになったため、日齢22に手術を行った。胸骨正中切開にて開胸し、胸腺を温存して腫瘤を切除した。病理検査にて右葉の病変は成熟奇形腫であったが、左葉の病変は単房性胸腺嚢胞と診断され、奇形腫の穿破ではなかった。術後経過は良好である。前縦隔には様々な嚢胞状腫瘤が発生するが、両者ともに出生前に指摘されることはほとんどなく、さらに両者を合併した報告はなかった。(著者抄録)

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

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

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

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

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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   Publisher：日本成人先天性心疾患学会  

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CiNii Article

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Other Link： http://search.jamas.or.jp/link/ui/2021096481

J-GLOBAL

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J-GLOBAL

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

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

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

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

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

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

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

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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  

CiNii Article

CiNii Books

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Other Link： http://search.jamas.or.jp/link/ui/2020037540

J-GLOBAL

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

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

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Language：Japanese   Publisher：(株)文光堂  

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

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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   Publisher：日本成人先天性心疾患学会  

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J-GLOBAL

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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)日本心臓病学会  

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

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Language：Japanese   Publisher：(株)文光堂  

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Language：Japanese   Publisher：(NPO)日本呼吸器外科学会  

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Language：Japanese   Publisher：岡山医学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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

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

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

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

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Language：Japanese  

CiNii Article

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Other Link： http://search.jamas.or.jp/link/ui/2017078319

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

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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：(株)メディカル・サイエンス・インターナショナル  

先天性心疾患に対する外科治療は近年,目覚ましい成績向上がみられる一方,そうした患者が成人期を迎え,さまざまな問題を抱えるケースが増加している。特に単心室症に対するFontan手術は,従来"根治術"とされてきたが,この手術が始まってから30年が経過し,さまざまな遠隔期の合併症が顕在化するにつれ,"Fontan palliation"であることが明白になってきた。心機能低下,房室弁逆流,不整脈,蛋白漏出性胃腸症など,Fontan手術後に特有の合併症に加え,長期のFontan循環による多臓器不全も治療を進めていくうえで問題となる。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2016&ichushi_jid=J02927&link_issn=&doc_id=20160519280006&doc_link_id=10.11477%2Fmf.3101200562&url=https%3A%2F%2Fdoi.org%2F10.11477%2Fmf.3101200562&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：岡山医学会  

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J-GLOBAL

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J-GLOBAL

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J-GLOBAL

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Language：Japanese   Publisher：日本静脈学会  

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Language：Japanese   Publisher：日本静脈学会  

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

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

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

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

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

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J-GLOBAL

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J-GLOBAL

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J-GLOBAL

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

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

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

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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：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：Japanese   Publisher：(一社)日本消化器がん検診学会  

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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：(一社)日本循環器病予防学会  

【目的】肥満は動脈硬化の独立した危険因子であるが、頸動脈エコーで評価した動脈硬化の状態と腹部脂肪分布を考慮した肥満との関係についての知見は十分でない。今回、頸動脈内膜中膜複合体厚(Intima-media thickness:IMT)の肥厚の有無に対する内臓脂肪および皮下脂肪蓄積の関係について調査した。【方法】対象は1,089人(女性278人)。日本脳神経超音波学会のガイドライン(案)に従い、頸動脈エコーによりIMT肥厚の有無(プラーク形成の有無に関わらず、max-IMT 1.1mm以上は肥厚あり)を評価した。IMT肥厚の有無と、臍レベルのCT撮影による内臓脂肪面積(visceral fat area:VFA)、皮下脂肪面積(subcutaneous fat area:SFA)との関係について、多重ロジスティック回帰分析を用いて性、年齢、収縮期血圧、脈拍数、LDLコレステロール、HDLコレステロール、中性脂肪、随時血糖で調整の上評価した。【結果】IMT肥厚あり群は760人、IMT肥厚なし群は335人であった。VFAはIMT肥厚あり群(110.1±51.9cm2)がIMT肥厚なし群(102.9±53.4cm2)より有意に高値であり(p=0.0368)、SFAはIMT肥厚あり群(154.3±59.1cm2)がIMT肥厚なし群(163.7±72.9cm2)より有意に低値であった(p=0.0260)。交絡要因で調整したところ、VFAの1SDあたりのオッズ比、95%信頼区間、p値は(1.23,1.03-1.47,p=0.0260)であり、SFAは(0.82,0.70-0.95,p=0.0104)であった。【結語】IMT肥厚に対して、内臓脂肪蓄積は促進的、皮下脂肪蓄積は抑制的であることが示唆された。(著者抄録)

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

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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)日本血管外科学会  

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

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

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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：Japanese   Publisher：シュプリンガー・ジャパン(株)  

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Language：Japanese   Publisher：シュプリンガー・ジャパン(株)  

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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：(一社)日本透析医学会  

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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)日本心臓血管外科学会  

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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：膜型人工肺研究会  

49歳男.胸部圧迫感,呼吸困難が出現した.胸部X線・心電図より急性心筋梗塞による急性左心不全を疑われた.画像所見により前壁から中隔領域にわたる広範囲の陳旧性梗塞に新たな切迫心筋梗塞が加わっていると考え,緊急冠動脈バイパス手術を施行した.吻合終了後,LOSのために人工心肺離脱不能と判断し,左房脱血-大動脈送血による左心バイパスを導入し人工心肺を離脱した.IABPは上室性不整脈が頻発するため効果的に駆動せず,経食道心エコーにてARの増悪も認め,OFFした.VT,Vf等の致死性不整脈を認めるようになり,左心バイパスからPCPSへの移行を行い,ICD埋め込み術を施行した.術後13日目,カテコラミンを増量し補助循環を離脱した.術後128日目,独歩退院となった

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Language：Japanese   Publisher：シュプリンガー・ジャパン(株)  

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Language：Japanese   Publisher：シュプリンガー・ジャパン(株)  

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Language：Japanese   Publisher：シュプリンガー・ジャパン(株)  

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Language：Japanese   Publisher：(一社)日本移植学会  

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

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Language：Japanese   Publisher：シュプリンガー・ジャパン(株)  

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

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

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

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

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Language：Japanese   Publisher：血管外科症例検討会  

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Language：Japanese   Publisher：シュプリンガー・ジャパン(株)  

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

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Language：Japanese  

CiNii Article

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Language：Japanese   Publisher：(一社)日本移植学会  

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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)日本血管外科学会  

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CiNii Article

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Language：Japanese   Publisher：岡山医学会  

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

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Language：Japanese   Publisher：福山市医師会  

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Language：Japanese   Publisher：岡山医学会  

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

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Language：Japanese   Publisher：シュプリンガー・ジャパン(株)  

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Language：Japanese   Publisher：広島医学会  

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Language：Japanese   Publisher：広島医学会  

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Language：Japanese   Publisher：社団法人日本循環器学会  

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Language：Japanese   Publisher：社団法人日本循環器学会  

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

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Language：Japanese   Publisher：岡山医学会  

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Language：Japanese   Publisher：金原出版(株)  

溶血性連鎖球菌(溶連菌)による軟部組織炎症の4症例を経験した.症例1は42歳男.右肩痛,背部痛,発熱が出現し,近医を受診したが,そこで血圧低下しプレショック状態となり,紹介入院となった.昇圧剤の投与及び抗ショック療法を行った.同時に抗菌薬としてFOM及びMINOを投与し,次いで,CEZやGCS-Fの投与も行った.後日,細菌培養よりA群溶連菌が同定された.症例2は50歳男.右足背の腫脹と発赤を認め受診した.壊死性筋膜炎と診断し,右足背の減張切開を施行した.後日,A群連鎖球菌を同定した.症例3は49歳男.左足関節外側が腫れ,痛みが出現し,壊死性筋膜炎の診断で入院となった.浮腫液の細菌培養の結果,検出されたのはA群溶連菌であった.症例4は69歳女.発熱,嘔吐により入院となった.抗ショック療法を開始した.細菌培養でC群連鎖球菌が同定された.症例1,4はTSLSと思われ,症例4は早期の診断・治療により治癒した

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Language：Japanese   Publisher：広島医学会  

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Language：Japanese   Publisher：広島医学会  

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

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

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Language：Japanese   Publisher：日本外科系連合学会  

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

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Language：Japanese   Publisher：岡山医学会  

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

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Language：Japanese   Publisher：(一社)国立医療学会  

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

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

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

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Language：Japanese   Publisher：岡山医学会  

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Language：Japanese   Publisher：岡山医学会  

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Language：Japanese   Publisher：岡山医学会  

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Event date： 2024.11.30 - 2024.12.1

Language：Japanese   Presentation type：Oral presentation (general)  

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Event date： 2024.11.8 - 2024.11.9

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Event date： 2024.11.1 - 2024.11.4

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Event date： 2024.11.1 - 2024.11.4

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Event date： 2024.11.1 - 2024.11.4

Language：Japanese   Presentation type：Oral presentation (general)  

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Event date： 2024.11.1 - 2024.11.4

Language：Japanese   Presentation type：Oral presentation (general)