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

Transcatheter closure of patent foramen ovale (PFO) is an effective strategy for preventing recurrence of paradoxical embolism. However, PFO closure is often associated with residual shunt, which is a risk of recurrent stroke. This study aimed to evaluate the relationship between the anatomical features of PFO and residual shunt. The degree of residual shunt and its relationship with the anatomical features of PFO were evaluated in 106 patients who underwent PFO closure at our institution between March 2011 and January 2022 and in whom contrast transthoracic echocardiography was performed 1 year later. The mean PFO tunnel length was 9.3 ± 3.6 mm and the mean PFO height was 3.2 ± 2.2 mm. Atrial septal aneurysm (ASA) was found in 37 patients. After PFO closure, residual shunt was observed in 28 patients (grade 1, n = 8; grade 2, n = 16; grade 3, n = 3; grade 4, n = 1). Univariate logistic analysis identified ASA to be associated with residual shunt (odds ratio 2.78, 95% confidence interval 1.14 to 6.79; p = 0.024). There was no association of residual shunt with the size of the PFO, the length of PFO tunnel, or the size of the device used for closure. Two of four patients with a large residual shunt of grade 3 or grade 4 were found to have device size mismatch. Residual shunt after PFO closure was observed in a quarter of patients and was related to the presence of ASA. A few patients had a large residual shunt due to the device size mismatch.

DOI： 10.1007/s12928-023-00979-y

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

DOI： 10.1016/j.jacadv.2024.100887

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

DOI： 10.1093/icvts/ivae033

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

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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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DOI： 10.1016/j.jscai.2023.101181

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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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DOI： 10.1016/j.jcin.2023.11.001

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We herein demonstrate three patients diagnosed with early hepatocellular carcinoma (HCC) during follow-up for Fontan-associated liver disease (FALD). Case 1: Twenty-one years after undergoing the Fontan procedure, a 26-year-old female was diagnosed with FALD. At the initial consultation, her serum alpha-fetoprotein (AFP) levels were markedly elevated, and dynamic enhanced computed tomography (CT) revealed HCC measuring 40 mm in diameter. She underwent partial hepatectomy. Ten months later, she underwent conventional transcatheter arterial chemoembolization (cTACE) for recurrent HCC near the resected hepatic stump as a curative treatment. Case 2: Twenty-one years after undergoing the Fontan procedure, a 25-year-old male was diagnosed with FALD and underwent HCC surveillance every 6 months. Thirteen months after the initial consultation, dynamic enhanced CT revealed HCC measuring 10 mm in diameter. He received cTACE as a curative treatment. Case 3. Twenty-eight years after undergoing the Fontan procedure, a 37-year-old male was diagnosed with FALD and underwent HCC surveillance every 3 months. Fourteen months later, abdominal ultrasonography (US) revealed HCC measuring 13 mm in diameter. He received radiofrequency ablation. These cases showed that HCC surveillance using abdominal US and AFP measurements in patients with FALD enables the detection of HCC and increases the chance of a cure.

DOI： 10.1007/s12328-023-01892-w

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DOI： 10.1016/j.amjcard.2023.07.125

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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

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BACKGROUND: Patent foramen ovale (PFO) is associated with various diseases such as cryptogenic stroke, migraine, and platypnea-orthodeoxia syndrome. This study aimed to evaluate the diagnostic performance of cardiac computed tomography (CT) for PFO detection. MATERIALS AND METHODS: Consecutive patients diagnosed with atrial fibrillation and who underwent catheter ablation with pre-procedural cardiac CT and transesophageal echocardiography (TEE) were enrolled in this study. The presence of PFO was defined as (1) the confirmation of PFO using TEE and/or (2) the catheter crossing the interatrial septum (IAS) into the left atrium during ablation. CT findings indicative of PFO included (1) the presence of a channel-like appearance (CLA) on the IAS and (2) a CLA with a contrast jet flow from the left atrium to the right atrium. The diagnostic performance of both a CLA alone and a CLA with a jet flow was evaluated for PFO detection. RESULTS: Altogether, 151 patients were analyzed in the study (mean age, 68 years; men, 62%). Twenty-nine patients (19%) had PFO confirmed by TEE and/or catheterization. The diagnostic performance of a CLA alone was as follows: sensitivity, 72.4%; specificity, 79.5%; positive predictive value (PPV), 45.7%; negative predictive value (NPV), 92.4%. The diagnostic performance of a CLA with a jet flow was as follows: sensitivity, 65.5%; specificity, 98.4%; PPV, 90.5%; NPV, 92.3%. The diagnostic performance of a CLA with a jet flow was statistically superior to that of a CLA alone (p = 0.045), and the C-statistics were 0.76 and 0.82, respectively. CONCLUSION: A CLA with a contrast jet flow in cardiac CT has a high PPV for PFO detection, and its diagnostic performance is superior to that of a CLA alone.

DOI： 10.3390/jcdd10050193

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DOI： 10.1007/s12928-022-00887-7

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UNLABELLED: A 46-year-old woman with a history of repeated thromboembolic stroke and anti-phospholipid antibody syndrome was referred to our hospital. Saline contrast transthoracic echocardiography showed that microbubbles appeared in the left atrium within 4 heartbeats. Thus, she was initially suspected as having a patent foramen ovale with associated paradoxical embolism. However, no evidence of patent foramen ovale or atrial septal defect could be found using transesophageal echocardiography. Saline contrast transesophageal echocardiography showed that microbubbles flowed into the left atrium through the left superior pulmonary vein. Ultimately, she was diagnosed as having a pulmonary arteriovenous malformation located at the upper left pulmonary lobe using contrast computed tomography and pulmonary artery angiography. Pulmonary arteriovenous malformations are typically located in the lower lobe of either lung and, in bubble studies, contrast appears in the left atrium after 4 heartbeats. Here, the pulmonary arteriovenous malformation was in the upper lobe, and contrast appeared in the left atrium at an earlier time point: one associated with patent foramen ovale. These findings made it difficult to differentiate the two diseases initially. This case suggests that pulmonary arteriovenous malformation should be carefully considered, even if microbubbles appear in the left atrium early on a saline contrast transthoracic echocardiograph. LEARNING OBJECTIVE: Pulmonary arteriovenous malformation occasionally appears in the upper lobe. In these cases, microbubbles may appear in the left atrium after detection in the right atrium with a time-course that is suggestive of a patent foramen ovale. Therefore, diagnosis should be carefully confirmed by using other multimodal imaging tests, such as transesophageal echocardiography, contrast computed tomography, or pulmonary artery angiography.

DOI： 10.1016/j.jccase.2022.11.005

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

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

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

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

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Cardiovascular diseases and liver diseases are closely related. Non-alcoholic fatty liver disease has the same risk factors as those for atherosclerotic cardiovascular disease and may also be a risk factor for atherosclerotic cardiovascular disease on its own. Heart failure causes liver fibrosis, and liver fibrosis results in worsened cardiac preload and congestion. Although some previous reports regard the association between cardiovascular diseases and liver disease, the management strategy for liver disease in patients with cardiovascular diseases is not still established. This review summarized the association between cardiovascular diseases and liver disease. In patients with non-alcoholic fatty liver disease, the degree of liver fibrosis progresses with worsening cardiovascular prognosis. In patients with heart failure, liver fibrosis could be a prognostic marker. Liver stiffness assessed with shear wave elastography, the fibrosis-4 index, and non-alcoholic fatty liver disease fibrosis score is associated with both liver fibrosis in patients with liver diseases and worse prognosis in patients with heart failure. With the current population ageing, the importance of management for cardiovascular diseases and liver disease has been increasing. However, whether management and interventions for liver disease improve the prognosis of cardiovascular diseases has not been fully understood. Future investigations are needed.

DOI： 10.3390/nu15030748

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DOI： 10.34376/jsachd.C-2022-0002

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BACKGROUND: Atrial septal defect (ASD) closure can cause acute pulmonary edema. Before transcatheter closure is performed, temporary balloon occlusion test (BOT) is recommended in patients with left ventricular dysfunction to predict the risk of pulmonary edema. However, the accuracy of BOT has not been verified. This study aimed to compare hemodynamic differences between BOT and transcatheter closure. METHODS: A total of 42 patients with a single ASD over age 18 years who underwent BOT before transcatheter ASD closure between October 2010 and May 2020 were analyzed. Pulmonary capillary wedge pressure (PCWP) was measured using a Swan-Ganz catheter placed in the pulmonary artery at baseline, after 10 min of BOT, and after transcatheter closure. Amplatzer septal occluder was used for all transcatheter closures. RESULTS: Mean patient age was 64 ± 18 years (range, 18-78). Mean ASD diameter and pulmonary to systemic flow ratio were 18 ± 5 and 2.8 ± 1.0 mm, respectively. Mean PCWP at baseline, during BOT, and after transcatheter closure was 8.9 ± 2.9, 13.5 ± 4.2, and 9.5 ± 2.6 mmHg, respectively. The difference between BOT and after transcatheter closure values was significant (p < 0.001). During BOT, PCWP increased ≥18 mmHg in 7 patients, whereas after ASD closure, PCWP was <18 mmHg in all 7 and none developed acute pulmonary edema. CONCLUSION: Temporary balloon occlusion of an ASD and transcatheter ASD closure result in different hemodynamic change. BOT overestimates increase of PCWP after transcatheter ASD closure and requires careful interpretation. Well-designed, larger studies in higher-risk patients are warranted to verify the clinical implications of BOT in more detail.

DOI： 10.1002/ccd.30556

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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)日本小児循環器学会  

心房中隔欠損(Atrial septal defect,ASD)は頻度の高い先天性心疾患であり,チアノーゼなどの症状が出ないことも多く,小児期に診断されることなく成人に到達する症例も少なくない.成人期まで到達したASDでは長年の右心系の負荷と肺血流の増加に伴い,心不全,心房細動,肺高血圧,などの合併症を伴い病態が複雑となることがある.従来の外科手術に加えて,近年の経カテーテルASD閉鎖術,心房細動に対するカテーテルアブレーション,肺高血圧治療薬の進歩に伴い治療成績は向上している.(著者抄録)

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J02003&link_issn=&doc_id=20230424230003&doc_link_id=10.9794%2Fjspccs.38.229&url=https%3A%2F%2Fdoi.org%2F10.9794%2Fjspccs.38.229&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

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BACKGROUND: Transcatheter mitral valve repair with the MitraClip system has been established in selected high-risk patients. The MitraClip procedure results in a relatively large iatrogenic atrial septal defect (iASD). This study aimed to investigate the prevalence and clinical course of iASD requiring transcatheter closure following the MitraClip procedure.Methods and Results: This study was conducted at all 59 institutions that perform transcatheter mitral valve repair with the MitraClip system in Japan. The data of patients on whom transcatheter iASD closure was performed were collected. Of the 2,722 patients who underwent the MitraClip procedure, 30 (1%) required transcatheter iASD closure. The maximum iASD size was 9±4 mm (range, 3-18 mm). The common clinical course of transcatheter iASD closure was hypoxemia with right-to-left shunt or right-sided heart failure with left-to-right shunt. Of the 30 patients, 22 (73%) required transcatheter closure within 24 h following the MitraClip procedure, including 12 with hypoxemia and 5 with right-sided heart failure complicated with cardiogenic shock. Of the 5 patients, 2 required mechanical circulatory support devices. Twenty-one patients immediately underwent transcatheter iASD closure, and hemodynamic deteriorations were resolved; however, 1 patient died without having undergone transcatheter closure. CONCLUSIONS: Transcatheter iASD closure was required in 1% of patients who underwent the MitraClip procedure. Many of these patients immediately underwent transcatheter iASD closure because of hypoxemia with right-to-left shunt or right-sided heart failure with left-to-right shunt.

DOI： 10.1253/circj.CJ-22-0048

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Language：Japanese   Publisher：(公財)日本心臓財団  

43歳男性.非代償性アルコール性肝硬変のため他院で通院加療を行われていた.特発性細菌性腹膜炎を契機とした肝腎症候群による急性腎障害を発症し当院転院となった.著明なアシドーシスおよび腎不全を認め集中治療室に入室した.入院後,下壁誘導ST上昇および高度房室ブロックを生じショック状態に至った.気管挿管を行いアルブミン補充および昇圧薬を使用して呼吸循環を維持し,持続的血液濾過透析を施行しながら緊急冠動脈造影検査を施行した.右冠動脈中間部90%狭窄,左冠動脈前下行枝近位部90%狭窄,左冠動脈回旋枝中間部90%狭窄を認めたが,血管拡張薬冠注により狭窄は解除され冠攣縮に伴うST上昇型急性下壁心筋梗塞と診断した.ニコランジル持続静脈投与を開始しST変化や房室ブロックの再発を認めなかった.血行動態安定し,全身状態も改善に向かった.肝腎症候群は末期肝硬変に続発する腎皮質血管の攣縮により生じるとされ,肝・腎以外の臓器にも血流障害が併存する可能性を示唆されている.今回我々は肝腎症候群に冠攣縮による急性心筋梗塞を発症し,集学的治療により救命し得た1例を経験したためここに報告する.(著者抄録)

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J00679&link_issn=&doc_id=20221018180015&doc_link_id=%2Fah2sinzd%2F2022%2F005410%2F019%2F1164-1169%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fah2sinzd%2F2022%2F005410%2F019%2F1164-1169%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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DOI： 10.1253/circj.CJ-22-0134

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The 86thAnnual Scientific Meeting of the Japanese Circulation Society was held in a web-based format on March 11-13, 2022. In accordance with the internationalization policy of the JCS, the meeting was held with the Asian Pacific Society of Cardiology Congress 2022. The main theme was "Cardiology Spreading its Wings". The number of patients with heart failure and other cardiovascular diseases is increasing dramatically, and the fields dealt with by cardiovascular medicine are also greatly expanding. This conference was both intellectually satisfying and exciting for all participants, who numbered over 14,900. The meeting was completed with great success, and the enormous amount of cooperation and support from all involved was greatly appreciated.

DOI： 10.1253/circj.CJ-22-0349

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

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OBJECTIVE: Therapeutic strategies for atrial septal defect (ASD) with severe pulmonary arterial hypertension (PAH) are controversial. This study aimed to evaluate the efficacy of PAH-specific medications and subsequent transcatheter closure (ie, treat-and-repair strategy) on clinical outcomes. METHODS: We enrolled 42 patients who were referred to 13 institutions for consideration of ASD closure with concomitant PAH and underwent the treat-and-repair strategy. The endpoint was cardiovascular death or hospitalisation due to heart failure or exacerbated PAH. RESULTS: At baseline prior to PAH-specific medications, pulmonary to systemic blood flow ratio (Qp:Qs), pulmonary vascular resistance (PVR), and mean pulmonary artery pressure (PAP) were 1.9±0.8, 6.9±3.2 Wood units and 45±15 mm Hg. Qp:Qs was increased to 2.4±1.2, and PVR and mean PAP were decreased to 4.0±1.5 Wood units and 35±9 mm Hg at the time of transcatheter ASD closure after PAH-specific medications. Transcatheter ASD closure was performed without any complications. During a median follow-up period of 33 months (1-126 months) after transcatheter ASD closure, one older patient died and one patient was hospitalised due to heart failure, but the other patients survived with an improvement in WHO functional class. PAP was further decreased after transcatheter ASD closure. CONCLUSIONS: The treat-and-repair strategy results in low complication and mortality rates with a reduction in PAP in selected patients with ASD complicated with PAH who have a favourable response of medical therapy.

DOI： 10.1136/heartjnl-2021-319096

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Transcatheter closure of patent foramen ovale (PFO) is an effective therapy for preventing recurrent stroke in very specific patient cohorts, such as cryptogenic stroke (CS). The identification of high-risk PFO, which is more likely to be linked to CS, is essential. This study aimed to assess the accuracy of saline contrast transthoracic echocardiography (TTE) for evaluating large right-to-left (RL) shunt. We enrolled 119 patients with or without CS who were confirmed to have PFO by transesophageal echocardiography (TEE) or catheterization. The severity of RL shunt evaluated by TTE and TEE was classified as follows: small (< 10 microbubbles), moderate (10-20 microbubbles), and large (> 20 microbubbles). With TTE, large RL shunt was observed in 94 (79%) of 119 patients, including 66 of 74 with CS and 28 of 45 without CS. With TEE, large RL shunt was observed in 33 (28 %) patients, including 26 with CS and 7 without CS. TTE showed large RL shunt more frequently than TEE (p < 0.01). Large RL shunt evaluated by TTE had a sensitivity of 89 % and an accuracy of 70 % for the association with CS, whereas large RL shunt evaluated by TEE had a sensitivity of 35% and an accuracy of 56 %. Accuracy was significantly greater in TTE than in TEE (p = 0.02). In conclusion, TTE identified large RL shunt associated with CS with higher sensitivity and accuracy compared to TEE. Our findings suggest that the decision for device closure should be made based on the severity of RL shunt by TTE.

DOI： 10.1007/s10554-021-02418-6

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

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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：(一社)日本成人先天性心疾患学会  

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BACKGROUND: Noninvasive assessment of stenotic lesions in patients with complex adult congenital heart disease (ACHD) is challenging due to its complex morphology. The simultaneous two-screen display of multidetector-computed tomography (MDCT) and real-time echogram (STDME) technology can display a virtual multi-planar reconstruction from MDCT corresponding to the same cross-sectional image from transthoracic echocardiography (TTE). We investigated the usefulness of the STDME technology for stenosis severity assessment in complex ACHD patients. METHODS: Twenty-four complex ACHD patients with stenotic lesions were enrolled in this study. All patients underwent TTE and the STDME technology within a week after MDCT. Peak velocity and pressure gradient (PG) across the stenotic site were measured using continuous wave Doppler. Cardiac catheterization was performed in 17 patients. RESULTS: Nine out of the twenty-four patients had undergone repair with a conduit. Peak velocity and PG from the STDME technology were higher than those from TTE (peak velocity: 3.1 ± 1.1 vs. 2.8 ± 1.0 m/s; peak PG: 43 ± 28 vs. 34 ± 21 mmHg; both p < 0.01). Peak PG from the STDME technology showed significant correlations with those from catheterization in patients with a conduit (n=7) and those without a conduit (n=10) (r = 0.795 and 0.880, respectively; both p < 0.05), while peak PG from TTE was correlated with catheterization measurements only in patients without a conduit (r = 0.850, p < 0.05). CONCLUSIONS: The STDME technology enables more accurate assessment of conduit stenosis severity than does TTE in complex ACHD patients.

DOI： 10.1016/j.jjcc.2021.06.008

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

DOI： 10.1007/s12574-021-00533-w

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BACKGROUND: Because transcatheter closure of patent foramen ovale (PFO) has become effective for preventing cryptogenic stroke (CS), it is necessary to determine high-risk PFO associated with CS. This study aimed to clarify the importance of direct right-to-left (RL) shunt through the PFO for identifying high-risk PFO. METHODS: We analyzed 137 patients with and without CS who were confirmed to have PFO. The timing of RL shunt through the PFO was evaluated by cardiac cycles after right atrium (RA) opacification on saline contrast transesophageal echocardiography. Direct RL shunt was defined as microbubbles crossing the PFO before and at the same time of RA opacification. RESULTS: Cardiac cycles of microbubbles crossing the PFO were shorter in patients with CS than in those without CS (2.0 ± 2.2 vs .5 ± 1.1, p < 0.01). Direct RL shunt was more frequently observed in patients with CS than in those without CS (77% vs 29%, p < 0.01), with a sensitivity of 79% and a specificity of 71% for the association with CS. Multivariate analysis revealed that direct RL shunt was related to atrial septal aneurysm and low-angle PFO. Regarding functional features of PFO, the detection rate of CS was 50% for large RL shunt alone, and was increased to 83% when direct RL shunt was added. CONCLUSION: Direct RL shunt was associated with CS and had the incremental value in detecting PFO associated with CS for large RL shunt. The timing of RL shunt can be valuable for identifying high-risk PFO.

DOI： 10.1111/echo.15234

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BACKGROUND/OBJECTIVES: Pregnancy may potentiate the inherent hypercoagulability of the Fontan circulation, thereby amplifying adverse events. This study sought to evaluate thrombosis and bleeding risk in pregnant women with a Fontan. METHODS: We performed a retrospective observational cohort study across 13 international centres and recorded data on thrombotic and bleeding events, antithrombotic therapies and pre-pregnancy thrombotic risk factors. RESULTS: We analysed 84 women with Fontan physiology undergoing 108 pregnancies, average gestation 33±5 weeks. The most common antithrombotic therapy in pregnancy was aspirin (ASA, 47 pregnancies (43.5%)). Heparin (unfractionated (UFH) or low molecular weight (LMWH)) was prescribed in 32 pregnancies (30%) and vitamin K antagonist (VKA) in 10 pregnancies (9%). Three pregnancies were complicated by thrombotic events (2.8%). Thirty-eight pregnancies (35%) were complicated by bleeding, of which 5 (13%) were severe. Most bleeds were obstetric, occurring antepartum (45%) and postpartum (42%). The use of therapeutic heparin (OR 15.6, 95% CI 1.88 to 129, p=0.006), VKA (OR 11.7, 95% CI 1.06 to 130, p=0.032) or any combination of anticoagulation medication (OR 13.0, 95% CI 1.13 to 150, p=0.032) were significantly associated with bleeding events, while ASA (OR 5.41, 95% CI 0.73 to 40.4, p=0.067) and prophylactic heparin were not (OR 4.68, 95% CI 0.488 to 44.9, p=0.096). CONCLUSIONS: Current antithrombotic strategies appear effective at attenuating thrombotic risk in pregnant women with a Fontan. However, this comes with high (>30%) bleeding risk, of which 13% are life threatening. Achieving haemostatic balance is challenging in pregnant women with a Fontan, necessitating individualised risk-adjusted counselling and therapeutic approaches that are monitored during the course of pregnancy.

DOI： 10.1136/heartjnl-2020-317397

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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OBJECTIVES: Patent foramen ovale is a hemodynamically insignificant interatrial communication that may cause ischemic stroke. Percutaneous patent foramen ovale closure reduces the risk for recurrent ischemic stroke in patients with a history of cryptogenic ischemic stroke. This study evaluated the cost-effectiveness of patent foramen ovale closure against medical therapy in patients after their first cryptogenic ischemic stroke in Japan. MATERIALS AND METHODS: The cost-effectiveness of patent foramen ovale closure compared with medical therapy was evaluated using the Markov model. The target patients started with patent foramen ovale closure or medical therapy for preventing secondary ischemic stroke under a stable state. Quality-adjusted life year was used as the outcome of effectiveness, and the analysis was conducted with a discount rate of 2% applied to both cost and effectiveness. The results of a multicenter open-label randomized controlled trial (RESPECT trial) evaluating patent foramen ovale closure using the Amplatzer™ PFO Occluder were used as clinical evidence. Cost-effectiveness was evaluated using the incremental cost-effectiveness ratio. It was evaluated as cost-effective if it was lower than 5 million JPY/ quality-adjusted life year. RESULTS: Patent foramen ovale closure was dominant over medical therapy by 2.53 quality-adjusted life years and an estimated cost reduction of 2,353,926 JPY. The probability of patent foramen ovale closure being dominant was 82.9%. CONCLUSIONS: Patent foramen ovale closure was dominant over medical therapy for preventing secondary ischemic stroke in patients with cryptogenic ischemic stroke.

DOI： 10.1016/j.jstrokecerebrovasdis.2021.105884

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• Identification of high-risk PFO associated with stroke is important. • Low-angle PFO is reported to be one of the high-risk PFO morphologies. • Low-angle PFO can allow venous blood to flow directly from the IVC to the LA. • The case showed direct blood flow from the IVC to LA through the low-angle PFO.

DOI： 10.1016/j.case.2021.02.008

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Few studies have reported the efficacy of Occlutech Figulla Flex II (FFII) device compared with Amplatzer Septal Occluder (ASO) device. The aim of this study was to examine the efficacy and safety of FFII compared with ASO for transcatheter atrial septal defect (ASD) closure. We retrospectively evaluated 190 patients using FFII and 190 patients using ASO who underwent transcatheter ASD closure. ASD characteristics were evaluated by transesophageal echocardiography. The prevalence of procedural complications, including erosion, device embolization, stroke, and new-onset atrial arrhythmia, and the presence of a residual shunt were evaluated between the two groups during 12-month follow-up. FFII was used more frequently than ASO in patients with a deficient aortic rim or septal malalignment (P = 0.02, P < 0.01, respectively). The procedural complications of erosion, device embolization, and stroke did not occur in any patients. New-onset atrial arrhythmia occurred in 3 patients of the FFII group and 4 patients of the ASO group, and the difference between the two groups was not significant (P = 0.70). A large residual shunt (≥ 3 mm) was observed in 6 patients of the FFII group and 5 patients of the ASO group, and the difference between the two groups was not significant (P = 0.76). FFII was used frequently in patients with high-risk ASD morphology; however, there was no difference in the prevalence of procedural complications or efficacy between patients using FFII and those using ASO.

DOI： 10.1007/s00380-020-01739-1

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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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Language：Japanese   Publisher：日本肺高血圧・肺循環学会・日本小児肺循環研究会  

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Patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions but to date only one official position paper related to left circulation thromboembolism has been published. This interdisciplinary paper, prepared with the involvement of eight European scientific societies, reviews the available evidence and proposes a rationale for decision making for other PFO-related clinical conditions. In order to guarantee a strict evidence-based process, we used a modified grading of recommendations, assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements were weighed and graded according to predefined scales. Despite being based on limited and observational or low-certainty randomised data, a number of position statements were made to frame PFO management in different clinical settings, along with suggestions for new research avenues. This interdisciplinary position paper, recognising the low or very low certainty of existing evidence, provides the first approach to several PFO-related clinical scenarios beyond left circulation thromboembolism and strongly stresses the need for fresh high-quality evidence on these topics.

DOI： 10.1093/eurheartj/ehaa1070

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OBJECTIVES: This study aimed to assess the feasibility of transcatheter atrial septal defect (ASD) closure in patients with absent aortic rim. BACKGROUND: The indication of transcatheter closure for ASD with absent aortic rim is controversial. METHODS: We enrolled 547 patients with ASD who were scheduled for transcatheter closure. Morphologies of aortic rim were evaluated using transesophageal echocardiography (TEE). RESULTS: Aortic rim of <5 mm was observed in 396 (72%) patients; 128 (23%) had absent aortic rim of 0 mm, and 268 (49%) had deficient aortic rim of >0 to <5 mm. Patients with absent aortic rim frequently had aortic rim absence at an angle of 0° on TEE and septal malalignment. Of the 128 patients with absent aortic rim, 126 (98%) successfully underwent transcatheter closure, while 2 (2%) failed transcatheter closure due to a large defect with severe septal malalignment. The success rate of transcatheter closure was similar between patients with absent aortic rim and those with deficient aortic rim (98% vs. 99%, p = .45). After the procedure, no patients had erosion or device embolization during a median follow-up of 24 months. CONCLUSIONS: Transcatheter closure was successfully performed without adverse events in patients with absent aortic rim, as well as in those with deficient aortic rim. Our findings can be valuable to determine the indication of transcatheter closure in patients with ASD.

DOI： 10.1002/ccd.29457

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Residual atrial septal defect (ASD) after surgical closure is rare, but some cases are seen during follow-up period. Redo surgery for residual ASD is often declined, while percutaneous closure can be acceptable. However, the indication of percutaneous closure for residual ASD has not been established. We reviewed our experience with percutaneous closure of residual ASD using medical and procedural records. Between 2006 and 2018, residual ASD was seen in seven patients. The median age of patients was 66 years (range 50-81 years), and the median period after surgical closure of ASD was 39 years (range 13-48 years). All patients had symptoms related to ASD. Percutaneous closure of residual ASD was successfully performed in all seven patients. No residual shunts were detected during the median follow-up period of 4.2 years (range 0.5-11 years) after percutaneous closure. Based on transesophageal echocardiographic findings and operative records for surgical closure, we considered two mechanisms causing residual ASD, such as the tear of surgical suture line and the overlooking of defect during surgical closure. Percutaneous closure of residual ASD was safely performed without any complications, suggesting that percutaneous is an effective therapeutic strategy for residual ASD after surgical closure.

DOI： 10.1007/s12928-020-00671-5

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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OBJECTIVES: This study aimed to determine morphological characteristics of deficient posterior-inferior rim for transcatheter atrial septal defect (ASD) closure success. BACKGROUND: The feasibility of transcatheter closure of ASD with deficient posterior-inferior rim remains unclear. METHODS: Of 869 patients with ASD who were scheduled transcatheter closure, 121 with posterior-inferior rim of <5 mm were included. Posterior-inferior rim morphologies were evaluated by transesophageal echocardiography. RESULTS: One hundred six patients successfully underwent transcatheter closure, while 15 patients failed. These 15 patients had complete deficient posterior-inferior rim of 0 mm and/or a large defect of ≥38 mm. Multivariate logistic regression analysis showed that transcatheter closure failure was independently related to complete deficient posterior-inferior rim and a large defect of ≥38 mm. Incomplete deficient posterior-inferior rim of >0 to <5 mm was observed in 84 patients. All these patients successfully underwent transcatheter closure, except two patients with a large defect of ≥38 mm. Complete deficient posterior-inferior rim was observed in 37 patients. The frequency of complete deficient posterior-inferior rim was higher in patients who failed transcatheter closure (87% vs. 23%, p < .01), but transcatheter closure was performed successfully if the range of complete deficient rim was ≤30°. After the procedure, no adverse events occurred during a median follow-up of 24 months. CONCLUSIONS: Most patients with deficient posterior-inferior rim successfully underwent transcatheter closure. Transcatheter closure could be performed even in patients with complete deficient posterior-inferior rim if the range was partial. Our findings can help to identify candidates for transcatheter closure.

DOI： 10.1002/ccd.29182

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Recent prospective controlled studies have demonstrated that transcatheter closure of a patent foramen ovale (PFO) reduces recurrent stroke risk in select patients, especially in patients younger than 60 years with PFO and embolic-appearing infarct and where no other mechanism of stroke was identified. Detection of PFO depends on the intensity of the Valsalva maneuver, and not all PFOs can be diagnosed using transesophageal echocardiography. Transthoracic contrast echocardiography using abdominal compression during the Valsalva maneuver is an easy method that can increase the detection sensitivity of PFO shunt. PFO with two or more of the following factors is most likely considered a "high-risk PFO" and as such, has a significantly higher probability of cryptogenic stroke: (1) a long-tunnel PFO (≥10 mm in length), (2) atrial septal aneurysm and/or hypermobile interatrial septum, (3) prominent Eustachian valve or Chiari's network, (4) large right-to-left shunt at rest and during the Valsalva maneuver, and (5) low-angle PFO. In order to establish the benefit of catheter-based PFO closure as a safe and effective treatment in clinical practice, the degree of accuracy of PFO diagnosis and its long-term safety need to be confirmed.

DOI： 10.1016/j.jjcc.2020.09.005

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DOI： 10.1016/j.echo.2020.08.001

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

Recently published long-term data from randomized controlled trials have provided evidence for the prevention of recurrent embolic stroke of undetermined source by percutaneous closure of the patent foramen ovale. However, most data were obtained from Caucasian populations and evidence on patent foramen ovale closure in Asian-Pacific patients is limited. The relative paucity in clinical data from this population, as well as the fact that Asian-Pacific patients may have higher bleeding risks than Caucasians, complicates clinical decision-making. This document, resulting from a consensus meeting of Asian-Pacific clinical experts, states the consensus among these experts about how to treat Asian-Pacific patients who had an embolic stroke of undetermined source and have a patent foramen ovale, based on currently available evidence and expert opinions. In addition, uncertainties and the need for clinical data regarding patent foramen ovale closure for prevention of recurrent embolic stroke of undetermined source in general, and specifically for Asian-Pacific patients, are identified.

DOI： 10.1177/1747493020941658

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• BVR can be used in patients with PA-IVS to avert abnormal hemodynamics. • Restrictive RV physiology is a unique Doppler echocardiographic feature in patients undergoing BVR. • Although 1.5VR conversion from BVR relieves congestive symptoms, restrictive RV physiology is not diminished.

DOI： 10.1016/j.case.2020.08.002

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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

Authorship：Corresponding author   Language：Japanese   Publishing type：Research paper (scientific journal)  

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

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

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

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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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Authorship：Corresponding author   Language：Japanese   Publishing type：Research paper (scientific journal)  

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

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

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

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

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

DOI： 10.32604/CHD.2020.011579

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Owing to new surgical procedures and medications, more women who have undergone the Fontan procedure reach childbearing ages. We report five cases of pregnancy with Fontan circulation. Case 1 had subchorionic hematoma (SCH), fetal growth restriction (FGR), and preterm labor (PTL). She delivered a 1073 g infant via cesarean section at gestation week 28 because of hemorrhagic shock. Case 2 delivered 2142 g and 2232 g infants at gestation weeks 37 and 36, respectively. She had FGR, PTL, and postpartum hemorrhage (PPH). Case 3 had SCH, PTL, and heart failure. At 36 weeks, labor was induced and she delivered a 2546 g infant by vacuum extraction with epidural analgesia. Cases 4 and 5 resulted in miscarriage. All subjects experienced obstetrical complications. This report discusses pregnant women with Fontan circulation by focusing on affected Japanese women. <Learning objective: More women who have undergone the Fontan procedure reach childbearing ages due to the new surgical procedures and medications. We report five cases and all of them experienced obstetrical complications. Anticoagulation might be one of the causes of postpartum hemorrhage. Labor epidural analgesia could contribute to minimized dynamic circulatory changes during labor. This report can be the literature regarding pregnant women with Fontan circulation in Japan.>.

DOI： 10.1016/j.jccase.2019.12.005

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

Authorship：Corresponding author   Language：Japanese   Publishing type：Research paper (scientific journal)  

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

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

BACKGROUND: Although transthoracic echocardiography (TTE) may be useful for patent foramen ovale (PFO) screening, the optimal methodologies remain unclear. The aims of this study were to evaluate the efficacy of the abdominal compression Valsalva maneuver and identify the optimal cutoff value of microbubbles in contrast TTE for detecting PFO, compared with transesophageal echocardiography and catheterization as the reference. METHODS: One hundred thirty-four patients with cryptogenic stroke or migraine who had suspected PFO and underwent TTE and transesophageal echocardiography plus catheterization were enrolled. The sensitivity, specificity, and accuracy of TTE for PFO detection were analyzed according to different provocations (spontaneous Valsalva maneuver, abdominal compression Valsalva maneuver) and different cutoff values of microbubbles for a positive result (at least one microbubble, at least five microbubbles). RESULTS: Eighty patients had PFO confirmed by transesophageal echocardiography and catheterization. When the cutoff was at least one microbubble, the sensitivity of TTE in detecting PFO was 93% with the spontaneous Valsalva maneuver and 99% with the abdominal compression Valsalva maneuver. When the cutoff was at least five microbubbles, sensitivity was 85% with the spontaneous Valsalva maneuver and 99% with the abdominal compression Valsalva maneuver. With the abdominal compression Valsalva maneuver, specificity was increased using the cutoff of at least five microbubbles compared with at least one microbubble (89% vs 57%). The abdominal compression Valsalva maneuver with the cutoff of at least 5 microbubbles provided the greatest accuracy of 95%. CONCLUSIONS: TTE with the abdominal compression Valsalva maneuver had excellent sensitivity. The cutoff of at least five microbubbles increased specificity. Our findings suggest that TTE with these criteria is valuable for PFO diagnosis.

DOI： 10.1016/j.echo.2019.09.018

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

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

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

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

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

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

Authorship：Corresponding author   Language：Japanese   Publishing type：Research paper (scientific journal)  

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：English   Publisher：日本成人先天性心疾患学会  

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Authorship：Corresponding author   Language：Japanese   Publishing type：Research paper (scientific journal)  

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BACKGROUND: Septal malalignment is related to erosion and device embolization in transcatheter closure of atrial septal defect (ASD), but limited information is available. OBJECTIVES: This study aimed to assess clinical significance of septal malalignment and to determine appropriate evaluation of ASD diameter, including the selection of device size. METHODS: Four hundred and seventeen patients with ASD who underwent transcatheter closure were enrolled. Septal malalignment was defined as separation between the septum primum and the septum secundum on transesophageal echocardiography. RESULTS: One hundred and eighty-four patients had septal malalignment. The frequency of septal malalignment increased with age reaching around 50% in adult patients. Septal malalignment was related to aortic rim deficiency. The distance of separation between the septum primum and the septum secundum was 5 ± 2 mm (range, 1-11 mm). In patients with septal malalignment, the ASD diameter measured at the septum primum was 19 ± 6 mm, while the ASD diameter measured at the septum secundum was 16 ± 6 mm. There was a difference of 4 ± 2 mm (range, 0-8 mm) between the ASD diameter measured at the septum primum and that measured at the septum secundum. For transcatheter closure, the Amplatzer Septal Occluder device size 2-3 mm larger and the Occlutech Figulla Flex II device size 4-7 mm larger than the ASD diameter measured at the septum primum were frequently used. During the study period, erosion or device embolization did not occur in all of the patients. CONCLUSIONS: Septal malalignment is highly prevalent in adult patients with aortic rim deficiency. The measurement of ASD diameter at the septum primum can be valuable for the selection of device size in patients with septal malalignment.

DOI： 10.1155/2020/6090612

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OBJECTIVE: The aim of this study was to examine the relationship between right ventricular (RV) volume and exercise capacity in adult patients with atrial septal defect (ASD) and to determine the degree of RV dilatation for transcatheter ASD closure. BACKGROUND: RV dilatation is an indication of transcatheter ASD closure; however, few studies have reported the clinical significance of RV dilatation. METHODS: We enrolled 82 consecutive patients (mean age, 49 ± 18 years; female, 68%) who underwent cardiac magnetic resonance imaging and symptom-limited cardiopulmonary exercise test before ASD closure. The relationship between RV volume and peak oxygen uptake (VO2) was evaluated. RESULTS: The mean RV end-diastolic volume index was 108 ± 27 ml/m2 (range, 46 to 180 ml/m2). The mean peak VO2 was 24 ± 7 ml/min/kg (range, 14 to 48 ml/min/kg), and the mean predicted peak VO2 was 90 ± 23%. There were significant negative relationships of RV end-diastolic volume index with peak VO2 (r = -0.28, p < 0.01) and predicted peak VO2 (r = -0.29, p < 0.01). The cutoff value of RV end-diastolic volume index <80% of predicted peak VO2 was 120 ml/m2, with the sensitivity of 49% and the specificity of 89%. CONCLUSIONS: There was a relationship between RV dilatation and exercise capacity in adult patients with ASD. RV end-diastolic volume index ≥120 ml/m2 was related to the reduction in peak VO2. This criterion of RV dilatation may be valuable for the indication of transcatheter ASD closure.

DOI： 10.1155/2020/9509105

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AIMS: There is no valid treatment strategy for addressing paroxysmal atrial fibrillation (AF) in patients with unclosed atrial septal defect (ASD). We aimed to assess the efficacy of catheter ablation (CA) compared with transcatheter ASD closure alone for treating pre-existing paroxysmal AF in patients with ASD. METHODS AND RESULTS: Among 908 patients who underwent transcatheter ASD closure, we evaluated 50 consecutive patients (63 ± 12 years) with paroxysmal AF. We compared the AF outcomes of these patients after transcatheter ASD closure between those with and without CA prior to ASD closure. Thirty (60%) patients underwent CA. During the follow-up period after ASD closure (mean: 49 ± 23 months), recurrence of AF was observed in 6/30 (20%) patients with upfront CA and 12/20 (60%) patients with ASD closure alone. Kaplan-Meier analysis showed that the AF-free survival rate was significantly higher for patients with CA than for those with ASD closure alone (79% vs. 37% at 5 years, P = 0.002). Upfront CA and previous heart failure hospitalization were associated with recurrence of AF after ASD closure [hazard ratio (HR) 0.18, 95% confidence interval (CI) 0.06-0.53; P = 0.002 and HR 4.64, 95% CI 1.60-13.49; P = 0.005, respectively]. CONCLUSION: In ASD patient with paroxysmal AF, transcatheter ASD closure alone demonstrated high AF recurrence rate after ASD closure. On the other hand, upfront CA prior to ASD closure substantially suppressed AF recurrence over the long term. A combination of CA and transcatheter ASD closure may be a feasible treatment strategy for paroxysmal AF in patients with ASD.

DOI： 10.1093/europace/euz207

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Oxford University Press  

The presence of a patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions
however, the subject remains controversial and no official statements have been published. This interdisciplinary paper, prepared with involvement of eight European scientific societies, aims to review the available trial evidence and to define the principles needed to guide decision making in patients with PFO. In order to guarantee a strict process, position statements were developed with the use of a modified grading of recommendations assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements of particular management options were weighed and graded according to predefined scales. Despite being based often on limited and non-randomised data, while waiting for more conclusive evidence, it was possible to conclude on a number of position statements regarding a rational general approach to PFO management and to specific considerations regarding left circulation thromboembolism. For some therapeutic aspects, it was possible to express stricter position statements based on randomised trials. This position paper provides the first largely shared, interdisciplinary approach for a rational PFO management based on the best available evidence.

DOI： 10.1093/eurheartj/ehy649

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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The follow-up of patients with adult congenital heart disease (ACHD) at a specialized medical unit is necessary for the patients' appropriate medical care. However, limited information is available about cardiovascular events among ACHD patients. Here we investigated the type and frequency of cardiovascular events in ACHD patients in relation to disease complexity. We retrospectively analyzed the cases of 535 patients (median age 35 years) referred to our ACHD center between 2014 and 2017. We divided the patients into 3 groups based on their disease complexity. To evaluate the relationship between disease complexity and cardiovascular events, we performed univariate and multivariate survival analyses. The Simple, moderate, and complex disease groups accounted for 62%, 19%, and 19% of the patients, respectively. Apart from events related to atrial septal defect (ASD) trans-catheter treatment, the frequency of cardiovascular events was dependent on the disease complexity (event-free survival rates at 3 years were 85%, 65%, and 58%, respectively). The hazard ratios were 4.0 and 5.1 in the moderate and complex groups, respectively. With the exception of scheduled transcatheter intervention, cardiovascular events were strongly related to the disease complexity of original heart disease. However, cardiovascular events were not rare even in the simple ACHD group.

DOI： 10.18926/AMO/56932

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BACKGROUND: Transcatheter closure of patent foramen ovale (PFO) has become an effective therapeutic strategy for cryptogenic stroke (CS). The identification of high-risk PFO is essential, but the data are limited. This study aimed to clarify the factors related to CS and to develop a score for high-risk PFO. METHODS: We retrospectively analyzed 57 patients with prior CS and 50 without CS who were scheduled for transcatheter closure. PFO characteristics were evaluated by transesophageal echocardiography. Based on factors related to CS, we estimated the risk score. RESULTS: Patients with CS had a greater frequency of large-size PFO (≥2 mm in height), long-tunnel PFO (≥10 mm in length), atrial septal aneurysm, hypermobile interatrial septum, prominent Eustachian valve or Chiari's network, the large right-to-left shunt at rest and during Valsalva maneuver, and low-angle PFO (≤10° of PFO angle from inferior vena cava), compared with patients without CS. Multivariate analysis showed that long-tunnel PFO, the presence of hypermobile interatrial septum, the presence of prominent Eustachian valve or Chiari's network, the large right-to-left shunt during Valsalva maneuver, and low-angle PFO were independently related to CS. When the score was estimated based on 1 point for each factor, the proportion of CS was markedly elevated with a score of ≥2 points. The probability of CS was markedly different between scores of ≤1 or ≥2 points. CONCLUSIONS: PFO risk can be assessed with a score based on high-risk features. The presence of two or more high-risk PFO features is associated with CS.

DOI： 10.1016/j.echo.2019.03.021

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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

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

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Language：English  

DOI： 10.1016/j.jcin.2018.10.057

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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：(一社)日本成人先天性心疾患学会  

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Language：English  

DOI： 10.1016/j.jcin.2018.06.055

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Japanese College of Cardiology (Nippon-Sinzobyo-Gakkai)  

Background: Management of adult congenital heart disease (ACHD) patients requires understanding of its complex morphology and functional features. An innovative imaging technique has been developed to display a virtual multi-planar reconstruction obtained from contrast-enhanced multidetector-computed tomography (MDCT) corresponding to the same cross-sectional image from transthoracic echocardiography (TTE). The aim of this study is to assess the usefulness of this imaging technology in ACHD patients. Methods: This study consisted of 46 consecutive patients (30 women
mean age, 52 ± 18 years old) with ACHD who had undergone contrast MDCT. All patients underwent TTE within a week of MDCT. An experienced sonographer who did not know the results of MDCT conducted a diagnosis using TTE and, then, using the new imaging technology. We studied whether this imaging technology provided additional or unexpected findings or makes more accurate diagnosis. Results: In this imaging technology, MDCT cross-section provides higher-resolution image to the deep compared to corresponding TTE image. Depending on the MDCT section which can be arbitrarily set under the echo guide, we can diagnose unexpected or incremental lesions or more accurately assess the severity of the lesion in 27 patients (59%) compared to TTE study alone. This imaging technology was useful in the following situations: 1. For anatomical guidance for Doppler flow assessment.2. Assessment of morphology of obstruction in outflow tracts or conduit.3. To clarify the etiology of unordinary severe valvular disease.4. Assessment of the patient with diseased prosthetic valves.Conclusions: This integrated imaging technology provides incremental role over TTE in complex anatomy, and allows functional information in ACHD patients.

DOI： 10.1016/j.jjcc.2018.04.015

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Language：English   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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

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

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

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

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

BACKGROUND: Fontan-associated liver disease (FALD) is an important late complication involving liver dysfunction, such as liver cirrhosis (LC) and hepatocellular carcinoma (HCC), in patients undergoing the Fontan procedure. However, the prevalence, clinical manifestation, and methods of diagnosis of FALD are still not well established.Methods and Results:This study comprised 2 nationwide surveys in Japan. First, the prevalence of LC and/or HCC in patients undergoing the Fontan procedure was determined. Second, clinical manifestations in patients with LC and/or HCC were analyzed, along with data from blood tests, echocardiography, and right heart catheterization. In the 1st survey, of the 2,700 patients who underwent the Fontan procedure, 31 were diagnosed with LC and/or HCC (1.15%), and 5 died due to liver diseases (mortality: 0.19%). In the 2nd survey, data were collected from 17 patients (12 with LC, 2 with HCC, and 3 with LC+HCC. Of these 17 patients, 5 died (mortality: 29.4%). The mean age at diagnosis of LC and HCC was 23 and 31 years, respectively. Computed tomography followed by ultrasound was most frequently used for diagnosis. Blood tests revealed low platelet counts, increased hemoglobin, aspartate aminotransferase, γ-guanosine triphosphate, and total bilirubin levels, and an elevated international normalized ratio of prothrombin time. CONCLUSIONS: LC and/or HCC in patients undergoing the Fontan procedure were not rare late complications and were associated with high mortality rates.

DOI： 10.1253/circj.CJ-17-1053

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

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

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Language：Japanese   Publisher：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   Publishing type：Research paper (scientific journal)   Publisher：Japanese Society of Internal Medicine  

Platypnea-orthodeoxia syndrome (POS) is a rare condition that is characterized by dyspnea and arterial oxygen desaturation, which worsen on standing and which are relieved by recumbency. We treated an 80-year-old woman with an atrial septal defect (ASD) who demonstrated POS following thoracic and lumbar vertebral compression fractures. The surgical closure of the ASD relieved her symptoms. The etiology might have been multiple compression fractures causing kyphosis and aortic distortion producing right atrial compression and increased right-to-left flow through the ASD. POS should be considered in the differential diagnosis of patients who develop dyspnea after vertebral compression fractures. The careful assessment of the patient’s history and clinical condition helps in the diagnosis of POS.

DOI： 10.2169/internalmedicine.9904-17

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Language：Japanese   Publisher：(NPO)日本冠疾患学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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

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

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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：(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)日本頭痛学会  

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Language：Japanese   Publisher：(一社)日本頭痛学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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Language：English   Publisher：(一社)日本心血管インターベンション治療学会  

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Language：Japanese   Publisher：(一社)日本頭痛学会  

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Language：Japanese   Publisher：(株)医学書院  

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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：(一社)日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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

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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：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本脈管学会  

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

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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Language：English   Publisher：ELSEVIER SCIENCE INC  

DOI： 10.1016/S0735-1097(13)61914-2

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Language：English   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)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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Language：English   Publisher：JAPANESE CIRCULATION SOC  

DOI： 10.1253/circj.CJ-88-0023

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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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Publishing type：Research paper (scientific journal)   Publisher：Japanese Society of Internal Medicine  

DOI： 10.2169/internalmedicine.51.6257

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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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)日本心臓病学会  

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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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)日本循環器学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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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)日本心臓血管外科学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：JAPANESE CIRCULATION SOC  

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 H) who underwent primary repair from September 1992 to December 2006. Median age and body weight at surgery were 21 days and 2.9kg, respectively. All but I 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, 19-8 1%) 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：Japanese   Publisher：(一社)日本心臓病学会  

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

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

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Language：English   Publishing type：Research paper (international conference proceedings)  

We analyzed the midterm results of children undergoing mitral valve repair without the use of prosthetic materials focusing on mitral annulus growth. From 1991 to 2004, 17 children (median age: 11 months) underwent mitral valve repair (grade III=9, IV=8). Regurgitation was due to prolapsed leaflet in 8 patients, annular dilatation in 4, and restrictive leaflet motion in 5. Preoperative indexed mitral valve diameter and Z-value were compared with those obtained at follow-up. There were no early or late deaths. All patients had an improved regurgitation grade after surgery. MV repair resulted in reduction in the indexed mitral valve diameter (58.2±22.9 vs. 47.3±18.9 mm/m 2, P&lt
0.05) and Z-value (3.3±2.3 vs. 0.79±2.2, P&lt
0.05). One patient underwent re-repair, but no patients required mitral valve replacement during the median follow-up period of 95 months. The latest regurgitation grade was absent or I in 4 patients, II in 10 patients, and III in 3 patients. Mitral valve annulus increased by 23% at 3 years and by 49% at 5 years compared with that at surgery. Mitral valve repair without the use of prosthetic materials is feasible for the majority of patients and carries an appropriate growth pattern of the mitral valve annulus after surgery. © 2006 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.

DOI： 10.1510/icvts.2005.125682

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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)日本小児循環器学会  

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

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

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

During past 10 years, the clinical experience of catheter interventional treatment in Kawasaki disease has been gradually increasing. These include balloon angioplasty, stent implantation, rotational ablation and transluminal coronary revascularization. Because the coronary artery stenosis in Kawasaki disease commonly involves severe calcification in contrast with adult atherosclerotic coronary artery lesions, the indication or technique of catheter intervention for adult patients cannot be directly employed in Kawasaki disease patients. Satisfactory acute results for coronary balloon angioplasty can be obtained for patients with a relatively short interval from the onset of disease, especially within 6 years
however, the incidence of restenosis after angioplasty is still high. Rotational ablation may be the most appropriate catheter intervention for Kawasaki disease. The advantage of rotational ablation is the high success rate, even in patients with calcified coronary artery stenosis. Stent implantation requires larger arterial access and is not possible in younger children. Care should be paid to the detection of new aneurysm formation, as the formation of new aneurysms is associated with the use of additional balloon angioplasty using high pressure balloon inflations. Anticoagulation or anti-platelet regimens are essential for the long-term management. © 2004 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.ppedcard.2004.08.010

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Language：English   Publisher：Japanese Circulation Society  

We surveyed Japanese experience of coil occlusion of patent ductus arteriosus up to 30 September 1996 by sending questionnaires to 175 hospitals. Thirty-four hospitals reported outcome data for 231 procedures in 218 patients. Successful implantation was achieved in 94% and acute complete closure of the ductus occurred in 71% of those in whom implantation of the coil was successful. Of the latter, 83% reported late complete closure. When those patients who underwent reocclusion for residual shunt are included, 89% attained complete closure. No life-threatening complications have occurred so far. Late reopening was reported in 3 cases. Although the angiographic type of ductus was significantly related to successful implantation (p<0.01), there was no significant correlation with complete occlusion. Ductuses with a minimum diameter greater than 3 mm had a decreased chance of successful implantation, whereas those less than 2 mm had a greater incidence of complete closure. (Jpn Circ J 1997; 61: 997 - 1003)

DOI： 10.1253/jcj.61.997

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Other Link： http://search.jamas.or.jp/link/ui/1998124670

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

Kawasaki disease (KD) is an acute systemic vasculitis syndrome of unknown etiology which mainly affects small and medium-sized arteries, particularly the coronary arteries. The long-term problems from KD are concerned almost entirely with the coronary arterial lesions that produce aneurysms and thrombotic occlusion and lead to premature atherosclerosis and ischemic heart disease. The disease was first recognized in Japan, but now it is found all over the world and is the leading cause of acquired heart disease in children in North America and Japan. It has been 25 years since KD was first reported and a large number of patients have become part of the pool of adults with ischemic heart disease. The long-term consequences and natural history of the cardiovascular involvements from KD remain uncertain. Since 1973 we have made a diagnosis of acute KD in 1664 patients and this cohort has been followed by coronary andiographic studies. From our study of these patients we will review the long-term issues regarding the evaluation and management of coronary artery disease caused by KD.

DOI： 10.1016/S1058-9813(97)00193-8

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

Aspirin is the basic treatment for Kawasaki disease, however its optimal dose is controversial. We investigated the therapeutic efficacy of high-dose (lOOmg/kg/day, n = 30) versus low-dose (30 mg/kg/day, n = 30) aspirin. Duration of fever, trasaminase, plasma thromboxane B2 (TXB2) and 6-keto-prostaglandin F1α (PGF1α) levels were compared before enrollment and on days 4, 7 and 14. In the high-dose group, duration of fever was significantly shorter than that of low-dose group (3.2±1.8 versus 5.4±4.3 days, p&lt
0.05), however, serum glutamic pyruvic transaminase levels were elevated (157.4 ± 187.7 versus 48.0± 58.21. U./liter, p&lt
0.005). No differences in the incidence of coronary artery lesions were observed (5 of 30 versus 7 of 30). Plasma TxB2 production was completely blocked in both groups, plasma 6-keto-PGF1α levels in the high-dose group on day 14 was lower than that in the low-dose group (39 α26 versus 160 α207 pg/ml, p&lt
0.05). This latter observation suggest that high-dose therapy may be disadvantageous as anti-thrombotic treatment, and supports the notion that low dose therapy is safe in the acute stage of Kawasaki disease. © 1990, Kurume University School of Medicine. All rights reserved.

DOI： 10.2739/kurumemedj.37.203

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Total pages：359p   Language：Japanese

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Responsible for pages：375-377   Language：Japanese Book type：Scholarly book

Responsible for pages：276-279   Language：Japanese

Responsible for pages：365-366   Language：Japanese Book type：Scholarly book

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Total pages：vii, 278p   Language：Japanese

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Total pages：287p   Language：Japanese

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Language：Japanese   Publisher：(公財)日本心臓財団  

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Language：Japanese   Publisher：(株)北隆館  

MitraClipや左心耳閉鎖のような経心房中隔穿刺によるカテーテル手技に伴い心房中隔に新たな欠損孔が出現することを医原性心房中隔欠損と呼ぶ。多くの医原性心房中隔欠損は無症候で自然閉鎖するといわれていたが,MitraClipに代表されるような重篤な心不全を基礎にもつ病態では,わずかな血行動態の変化が重篤な必不全進行への契機となる可能性がある,特に医原性心房中隔欠損を介して右左短絡が発生し,重篤な低酸素血症へと進行する症例では欠損孔の緊急的閉鎖術が必要となることがある。国内の現状とその対策について報告する。(著者抄録)

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Language：Japanese   Publisher：大道学館出版部  

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Language：Japanese   Publisher：(株)日本医事新報社  

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Language：Japanese   Publisher：(公財)日本心臓財団  

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Language：Japanese   Publisher：(株)日本医事新報社  

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Language：Japanese   Publisher：(一社)日本内科学会  

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

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

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Language：Japanese   Publisher：(一社)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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J-GLOBAL

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Language：Japanese   Publisher：(株)メジカルビュー社  

＜文献概要＞Point 1 循環器内科医にとって成人先天性心疾患診療は不可避の領域であり,特に中等症から重症先天性心疾患に対する診療体制構築を急ぐ必要がある。2 成人先天性心疾患総合修練施設を核として地域の診療ネットワークを構築し,地域格差のない診療体制の構築が望まれる。3 このような診療体制の中心を担う成人先天性心疾患専門医の養成が急務であり,専門医養成のための学会活動・教育活動が重要である。

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Language：Japanese   Publisher：(一社)日本未病学会  

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Language：Japanese   Publisher：(株)文光堂  

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Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞Point ・潜因性脳塞栓の再発予防に対するPFO閉鎖術には,高いエビデンスがある.・PFOの検出には,十分なValsalva負荷を用いた経胸壁バブルスタディーが重要である.・経食道心エコーではハイリスクPFOの評価が重要であり,治療適応判断に重要である.

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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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：English   Publishing type：Book review, literature introduction, etc.   Publisher：Europa Group  

Patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions but to date only one official position paper related to left circulation thromboembolism has been published. This interdisciplinary paper, prepared with the involvement of eight European scientific societies, reviews the available evidence and proposes a rationale for decision making for other PFO-related clinical conditions. In order to guarantee a strict evidence-based process, we used a modified grading of recommendations, assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements were weighed and graded according to predefined scales. Despite being based on limited and observational or low-certainty randomised data, a number of position statements were made to frame PFO management in different clinical settings, along with suggestions for new research avenues. This interdisciplinary position paper, recognising the low or very low certainty of existing evidence, provides the first approach to several PFO-related clinical scenarios beyond left circulation thromboembolism and strongly stresses the need for fresh high-quality evidence on these topics.

DOI： 10.4244/EIJ-D-20-00785

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

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Language：English  

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

DOI： 10.1016/j.jjcc.2020.01.008

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

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Language：Japanese   Publisher：(NPO)日本冠疾患学会  

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Language：Japanese   Publisher：(一社)日本脳卒中学会  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J01786&link_issn=&doc_id=20191001430012&doc_link_id=%2Fdh3strok%2F2019%2F004105%2F012%2F0417-0441%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdh3strok%2F2019%2F004105%2F012%2F0417-0441%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：Japanese   Publisher：(公財)日本心臓財団  

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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：ELSEVIER SCIENCE INC  

OBJECTIVES This study aimed to assess the fate of tricuspid regurgitation (TR) after transcatheter atrial septal defect (ASD) closure.& para;& para;BACKGROUND Although TR frequently occurs in patients with ASD, the change in TR during long-term follow-up after ASD closure remains unknown.& para;& para;METHODS A total of 419 adult patients who underwent transcatheter ASD closure were enrolled. TR severity was graded by TR jet area on echocardiography.& para;& para;RESULTS At baseline, 113 patients had severe/moderate TR and 306 patients had mild TR. Among the 113 patients with severe/moderate TR, the TR jet area significantly decreased during a median follow-up of 30 months after the procedure; this decrease was related to the improvement in right ventricular morphology. The severity of TR decreased to mild in 79 (70%) patients. Persistent TR, defined as severe or moderate TR after the procedure, was independently associated with the prevalence of permanent atrial fibrillation. Regarding clinical outcomes, 7 patients with severe/moderate TR and 2 with mild TR were hospitalized because of heart failure. Patients with severe/moderate TR had the worse event-free survival rate than those with mild TR, but more than 90 % of them had no cardiovascular events.New York Heart Association functional class and plasma B-type natriuretic peptide levels improved in patients with severe/moderate TR, similar to those with mild TR.& para;& para;CONCLUSIONS Significant TR decreased during the long-term follow-up period after transcatheter ASD closure. Heart failure symptoms improved in patients with severe/moderate TR. Our findings suggest that transcatheter closure alone can be valuable in patients with ASD complicated with TR. (C) 2017 by the American College of Cardiology Foundation.

DOI： 10.1016/j.jcin.2017.06.022

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：ELSEVIER SCIENCE INC  

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Language：English   Publisher：WILEY  

ObjectiveThis study evaluated the effect of echocardiographic left ventricular (LV) diastolic dysfunction on acute congestive heart failure after transcatheter atrial septal defect (ASD) closure in elderly patients.
BackgroundAlthough there is concern that LV diastolic dysfunction develops acute congestive heart failure after ASD closure, limited information is available regarding the influence, especially in elderly patients with severe LV diastolic dysfunction.
MethodsTwo hundred consecutive patients older than 60 years were divided into 3 groups according to echocardiographic LV diastolic dysfunction: severe (early diastolic mitral annular velocity [e'] &lt;5.0cm/s), mild (5.0 e'&lt;8.0cm/s), and normal (e'8.0cm/s). Changes in plasma B-type natriuretic peptide (BNP) levels were evaluated.
ResultsNo patients with severe LV diastolic dysfunction developed acute congestive heart failure immediately after the procedure. BNP levels unchanged after the procedure in patients with severe LV diastolic dysfunction (126181 to 131 +/- 148pg/ml, P=0.885), and this increase in BNP levels was not different from that between the diagnosis of ASD and the procedure. The change in BNP levels in patients with severe LV diastolic dysfunction, who were frequently treated with diuretics before the procedure, was equivalent to that in patients with mild LV diastolic dysfunction and normal LV diastolic function (5 +/- 119 vs. 16 +/- 101 vs. 9 +/- 131pg/ml, P=0.724).
ConclusionsOur findings suggest that transcatheter ASD closure under volume management is safe and valuable in elderly patients with echocardiographic severe LV diastolic dysfunction.

DOI： 10.1111/joic.12365

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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：(一社)日本超音波検査学会  

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Language：Japanese   Publisher：金原出版  

DOI： 10.18888/J00639.2017145609

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：ELSEVIER SCIENCE INC  

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

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Language：English   Publisher：Springer Tokyo  

Some types of migraine are associated with ischemic stroke. Although a right-to-left communication is linked with ischemic stroke, a causal relationship between migraine and right-to-left communication remains unclear. Furthermore, the efficacy of transcatheter closure of atrial communication on migraine is controversial. We aimed to evaluate the influence of transcatheter closure of atrial communication on migraine in patients with ischemic stroke. Thirty-eight consecutive patients with ischemic stroke who underwent transcatheter closure of atrial communication were enrolled. The prevalence, frequency, and severity of migraine were prospectively evaluated at baseline, 3 months, and &gt
6 months after the procedure. Changes in migraine after the procedure were classified according to the frequency and severity of migraine: disappeared, improved, no-change, and worsening. Nineteen (50 %) of 38 patients suffered from migraine at baseline. No significant differences were observed in age, comorbidities, defect diameter, and atrial septal aneurysm between patients with migraine and patients without migraine. Among the 19 patients with migraine, migraine disappeared in 10 (53 %) patients and improved in 8 (42 %) patients at 3 months after transcatheter closure of atrial communication. At mean follow-up of 38 ± 28 months after the procedure, migraine disappeared in 12 (63 %) patients and improved in five (26 %) patients. No patients experienced worsening of migraine during the follow-up period. New-onset migraine was not observed in patients without migraine. Migraine is complicated in a half of patients with ischemic stroke related to atrial communication. Such migraine may disappear or improve after transcatheter closure of atrial communication.

DOI： 10.1007/s12928-015-0375-8

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Language：English   Publisher：OKAYAMA UNIV MED SCHOOL  

A treatment strategy for patients with pulmonary hypertension (PH) and atrial septal defect (ASD) remains unclear. This study was designed to evaluate the effects of initial repair of ASD followed by treatment with PH-specific drugs in patients with PH and ASD. Eligible patients receive transcatheter ASD closure followed by treatment with bosentan and sildenafil. Right heart catheterization is performed at baseline and at 12, 24 and 48 weeks. The primary endpoint is change in pulmonary artery pressure and pulmonary vascular resistance from baseline to follow-up. This study should provide valuable information to establish a therapeutic strategy for PH and ASD.

DOI： 10.18926/AMO/54600

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Language：English   Publisher：MOSBY-ELSEVIER  

Background: The influence of deficient rims surrounding atrial septal defects (ASDs) in patients undergoing transcatheter closure has yet to be clarified. The aim of this study was to assess the influence of a deficient surrounding rim on the procedural success and clinical outcome of transcatheter ASD closure using an Amplatzer septal occluder.
Methods: A total of 474 patients (mean age, 46622 years) with ostium secundum ASDs measuring &lt;= 40 mm in diameter who had undergone attempted transcatheter closure using Amplatzer septal occluders from September 2007 to August 2013 were assessed. A comprehensive transesophageal echocardiographic examination was done to assess the morphologic characteristics of the defects in all patients. Subjects were classified into three groups by the extent and location of rim deficiency (&lt;5 mm): patients without deficient rims (sufficient group, n = 101), patients with single deficient rims, (single group, n = 338), and patients with multiple rim deficiencies (multiple group, n = 35).
Results: There was a significant difference in the maximal defect diameter among the sufficient, single, and multiple groups (15 +/- 6, 18 +/- 6, and 29 +/- 7 mm, respectively, P &lt; .001). Transcatheter closure was successfully accomplished in 463 patients (98%). The prevalence of procedural success differed significantly among the sufficient, single, and multiple groups (100%, 98%, and 86%, respectively, P &lt; .001). There was no significant difference in the occurrence of cardiovascular events among the three groups during a mean follow-up period of 25 +/- 19 months (P = .926, log-rank test).
Conclusions: In patients with ASDs with multiple rim deficiencies as determined by transesophageal echocardiography, successful transcatheter ASD closure using Amplatzer septal occluders is more difficult to accomplish. However, if closure is successful, rim deficiencies rarely affect intermediate-term outcomes.

DOI： 10.1016/j.echo.2016.04.010

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Language：English   Publisher：CAMBRIDGE UNIV PRESS  

An alternative approach for transcatheter closure of atrial septal defect is necessary in patients with absent inferior caval vein connection. In this report, we describe the successful transcatheter atrial septal defect closure via the transjugular approach using a steerable guide catheter.

DOI： 10.1017/S1047951116000445

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Language：English   Publisher：ELSEVIER SCIENCE INC  

DOI： 10.1016/j.jcin.2016.03.014

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

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：ELSEVIER SCIENCE INC  

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

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