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DOI： 10.1016/j.hrcr.2023.06.008

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

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BACKGROUND: J waves in the inferior or lateral leads are characteristic electrocardiographic (ECG) changes in patients with early repolarization syndrome (ERS). However, the presence of J waves in the left posterior region has not yet been evaluated. OBJECTIVE: The purpose of this study was to clarify the significance of J waves in the posterior left ventricle using leads V7-V9 and a body surface mapping (BSM) system. METHODS: Forty patients diagnosed with ERS were included. All patients exhibited J waves in either the contiguous inferior, lateral, or posterior leads. We evaluated the incidence of J waves in the inferolateral and posterior leads using a 15-lead ECG with synthesized V7-V9 and an 87-lead BSM. Additionally, we assessed the arrhythmogenicity of the posterior regions based on the morphology of the premature ventricular complexes (PVCs) associated with ventricular fibrillation (VF). RESULTS: J waves were observed in the lateral, inferior, and posterior leads of 26 (65%), 31 (78%), and 39 (97%) patients, respectively. J waves were found only in the posterior leads of 5 patients. BSM was evaluated in 9 patients, all of whom exhibited a positive area on the posterior region. PVCs associated with VF were recorded in 5 patients. Among patients with inferolateral and posterior J waves, all except 1 patient who displayed left bundle branch block morphology showed PVCs originating from the posterior left ventricular region. CONCLUSION: Posterior J waves are common in ERS patients. This abnormality can be detected using leads V7-V9 and the BSM system and may be associated with arrhythmogenesis.

DOI： 10.1016/j.hrthm.2023.08.032

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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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Background: Various types of arrhythmia are observed in patients with cardiac amyloidosis, but the prevalence of arrhythmia has not been fully investigated. This study investigated the prevalence and treatment of arrhythmias in patients with cardiac amyloidosis before the introduction of new agents for amyloidosis, such as tafamidis. Methods and Results: Of 53 patients who were histologically diagnosed with cardiac amyloidosis at 10 centers in western Japan between 2009 and 2021, 43 who were diagnosed on the basis of immunohistochemical staining were evaluated in this study. Of these 43 patients, 13 had immunoglobulin light-chain (AL) amyloidosis and 30 had transthyretin (ATTR) amyloidosis; further, 27 had atrial tachyarrhythmia, 13 had ventricular tachyarrhythmia, and 17 had bradyarrhythmia. Atrial fibrillation (AF) was the most common arrhythmia in patients with cardiac amyloidosis (n=24; 55.8%), especially among those with ATTR amyloidosis (70.0% of ATTR vs. 23.1% of AL). Eleven (25.6%) patients were treated with a cardiac implantable device. All 3 patients with pacemakers were alive at the last follow-up (median 76.7 months; interquartile range [IQR] 4.8-146.4 months). Of the 8 patients who underwent AF ablation, there was no recurrence in 6 (75%) after a median of 39.3 months (IQR 19.8-59.3 months). Conclusions: The prevalence of various arrhythmias was high in patients with cardiac amyloidosis. AF occurred most frequently in patients with cardiac amyloidosis, especially among patients with ATTR.

DOI： 10.1253/circrep.CR-23-0022

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Background The appearance of complete right bundle-branch block (CRBBB) in Brugada syndrome (BrS) is associated with an increased risk of ventricular fibrillation. The pathophysiological mechanism of CRBBB in patients with BrS has not been well established. We aimed to clarify the significance of a conduction delay zone associated with arrhythmias on CRBBB using body surface mapping in patients with BrS. Methods and Results Body surface mapping was recorded in 11 patients with BrS and 8 control patients both with CRBBB. CRBBB in control patients was transiently exhibited by unintentional catheter manipulation (proximal RBBB). Ventricular activation time maps were constructed for both of the groups. We divided the anterior chest into 4 areas (inferolateral right ventricle [RV], RV outflow tract [RVOT], intraventricular septum, and left ventricle) and compared activation patterns between the 2 groups. Excitation propagated to the RV from the left ventricle through the intraventricular septum with activation delay in the entire RV in the control group (proximal RBBB pattern). In 7 patients with BrS, excitation propagated from the inferolateral RV to the RVOT with significant regional activation delay. The remaining 4 patients with BrS showed a proximal RBBB pattern with the RVOT activation delay. The ventricular activation time in the inferolateral RV was significantly shorter in patients with BrS without a proximal RBBB pattern than in control patients. Conclusions The CRBBB morphology in patients with BrS consisted of 2 mechanisms: (1) significantly delayed conduction in the RVOT and (2) proximal RBBB with RVOT conduction delay. Significant RVOT conduction delay without proximal RBBB resulted in CRBBB morphology in patients with BrS.

DOI： 10.1161/JAHA.122.028706

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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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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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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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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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Constant pacing (S1-S1: 520 ms) from the right atrial appendage (RAA) during reentrant atrial tachycardia originating from the atrioventricular node vicinity. RA-FW 1-2 was constantly captured antidromically. However, the earliest atrial activation site (EAAS = His1-2) was orthodromically (red circle) and antidromically (blue square) captured at a ratio of 3:1. The electrogram morphology at the EAAS was different between orthodromically and antidromically electrograms captured during pacing. Moreover, the interval between the S1 and orthodromically captured electrogram was gradually prolonged.

DOI： 10.1002/joa3.12702

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Catheter ablation (CA) of ventricular tachycardia (VT) after repair of congenital heart disease may be difficult because of complex anatomy and sometimes unmappable VT. Here, we report a 41-year-old woman with successful CA of unmappable VT in a patient with complete transposition of the great arteries after Rastelli repair. Clinical VT was induced by programmed electrical stimulation, when the mapping catheter was placed at the high anterior right ventricular outflow tract (RVOT). During VT, the local potential at the high anterior RVOT under the right ventricle (RV) - pulmonary artery (PA) conduit was equal to that at the timing of onset of QRS. The VT was unmappable because the hemodynamics deteriorated. Pace mapping was also tried at the aortic cusp and the left ventricular outflow tract (LVOT). Fractionated potential during sinus rhythm was observed at the noncoronary cusp, and the paced QRS morphology at this site was similar to that of the clinical VT, with a delay of 55 ms from pacing to the onset of QRS. However, mapping at the LVOT was impossible due to the difficulty of catheter manipulation. Radiofrequency energy was successfully applied at the noncoronary cusp and the high anterior RVOT under the RV-PA conduit. <Learning objective: This report is a rare case of successful catheter ablation of unmappable ventricular tachycardia (VT) in a patient with complete transposition of the great arteries after Rastelli repair. The VT was unmappable because of intolerable hemodynamics. However, we could speculate the exit or isthmus of the VT by pace mapping or local potential and eliminate the VT.>.

DOI： 10.1016/j.jccase.2021.09.012

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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)日本成人先天性心疾患学会  

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Left atrial appendage (LAA) size is crucial for determining the indication of transcatheter LAA closure. The aim of this study was to evaluate the differences in LAA morphology according to the types of atrial fibrillation (AF). A total of 299 patients (mean age: 67 ± 13 years) who underwent transesophageal echocardiography (TEE) were included. Patients were classified into non-AF (n = 64), paroxysmal AF (n = 86), persistent AF (n = 87), or long-standing persistent AF (n = 62). LAA morphology, including LAA ostial diameter and depth, was assessed using TEE. Patients with long-standing persistent AF had larger LAA ostial diameter and depth and lower LAA flow velocity. The maximum LAA ostial diameter was 19 ± 4 mm in patients with non-AF, 21 ± 4 mm in patients with paroxysmal AF, 23 ± 5 mm in patients with persistent AF, and 26 ± 5 mm in patients with long-standing persistent AF. LAA ostial diameter was increased by 2 or 3 mm with the progression of AF. LAA ostial diameter was correlated with LA volume index (R = 0.37, P < 0.01) and the duration of continuous AF (R = 0.30, P < 0.01), but not with age or the period from the onset of AF. In conclusion, LAA size was increased with the progression of AF.

DOI： 10.1371/journal.pone.0278172

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BACKGROUND: Multiple spikes within the QRS complex, known as fragmented QRS (fQRS), are associated with the occurrences of ventricular arrhythmic events (VAEs) in patients with Brugada syndrome and hypertrophic cardiomyopathy. However, the association between fQRS and occurrence of VAEs in patients with cardiac sarcoidosis (CS) has not been elucidated. METHODS: We evaluated the associations between fQRS and cardiac events including VAEs [non-sustained ventricular tachycardia (NSVT), sustained ventricular tachycardia (VT), and ventricular fibrillation (VF)], hospitalization for heart failure, and all-cause death in 68 patients with CS (30 patients with fQRS vs. 38 patients without fQRS) over a 5-year period. RESULTS: Cardiac events occurred in 22 patients with fQRS and 18 patients without fQRS (73% vs. 47%, p=0.009). Of the cardiac events that occurred in CS patients, VAEs occurred more frequently in patients with fQRS than in patients without fQRS (VAEs: 70% vs. 45%, p=0.017; NSVT: 70% vs. 45%, p=0.010; VT: 43% vs. 18%, p=0.011, and VF: 6.7% vs. 2.6%, p=0.34), whereas there was no significant difference in hospitalization for heart failure or all-cause death between patients with and those without fQRS (hospitalization for heart failure: 6.7% vs. 5.3%, p=0.75; all-cause death: 6.7% vs. 5.3%, p=0.64). Multivariate analysis showed that fQRS in the baseline electrocardiogram was independently associated with VAEs (hazard ratio: 2.21, 95% confidence interval: 1.15-4.25, p=0.017). CONCLUSION: fQRS is a predictor of VAEs in patients with CS.

DOI： 10.1016/j.jjcc.2021.10.022

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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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DOI： 10.1016/j.hrcr.2021.07.008

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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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BACKGROUND: Numerous basic studies have shown a relationship between interleukin-6 (IL-6) and the development or severity of myocarditis. However, there has been no study in which the effect of IL-6 levels in patients with myocarditis was evaluated. METHODS: We enrolled control patients (n = 12) and consecutive patients with acute myocarditis (n = 13), including lymphocytic, eosinophilic, and giant cell myocarditis, and investigated the pathological and clinical effects of IL-6 on human myocarditis. RESULTS: The serum IL-6 level in patients with myocarditis (16.7 [9.9, 103.8] pg/mL) was significantly higher than that in the control patients (1.4 [1.0, 1.9] pg/mL) (P<0.001). Immunohistochemical analysis showed that IL-6 was expressed in infiltrating inflammatory cells of endomyocardial biopsy samples from all patients with myocarditis. Moreover, the log-transformed value of serum IL-6 level showed significant positive correlations with serum creatine kinase (CK) level, CK-MB level, peak CK level, peak CK-MB level and C-reactive protein level (all P ≤ 0.005) and a negative correlation with the left ventricular (LV) ejection fraction (p = 0.014). We divided the patients with myocarditis into a low IL-6 group (9.9 [4.5, 14.2] pg/dL, n = 7) and a high IL-6 group (108.9 [51.1, 130.9] pg/dL, n = 6). The degree of infiltration of IL-6-expressing inflammatory cells in myocardial samples obtained from patients in the high IL-6 group was significantly more severe than that in samples obtained from patients in the low IL-6 group. Furthermore, patients in the high IL-6 group significantly more frequently received catecholamine therapy (P = 0.005), venoarterial extracorporeal membrane oxygenation (P = 0.029), and artificial respirator support (P = 0.021) in the acute phase of myocarditis. CONCLUSION: The results suggest that there is a strong impact of IL-6 on cardiac injury and dysfunction in patients with myocarditis.

DOI： 10.1016/j.jjcc.2021.03.003

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The effectiveness of adenosine-induced flow arrest in surgical clipping for the cerebral aneurysms with difficulties in temporary clip placement to the proximal main trunk has been reported. This is the first clinical trial to evaluate the safety and feasibility of adenosine-assisted clipping surgery for unruptured cerebral aneurysms (UCAs) in Japan. The inclusion criteria are as follows: patients over 20 years old, patients who agree to be enrolled in this study after providing informed consent, patients who undergo clipping surgery for UCA in our institute, and patients in whom the surgeons (T.H. or I.D.) judge that decompression of the aneurysm is effective. The primary endpoint is a modified Rankin Scale (mRS) score 30 days after surgery. We plan to enroll 10 patients in this study. The original protocol of adenosine administration was established in this trial. Herein, we present the study protocol.

DOI： 10.2176/nmc.st.2021-0018

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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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• 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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BACKGROUND: Transvenous lead extraction (TLE) is necessary because of system infection, lead malfunction, or system upgrade. Patients with severe left ventricular dysfunction (SLVD) undergoing TLE may be at a higher risk because hemodynamic parameters may change unfavorably during or after TLE; however, this has not yet been clarified. OBJECTIVE: The purpose of this study was to examine whether patients with SLVD undergoing TLE have higher mortality. METHODS: All patients who underwent TLE were stratified as follows: patients with ejection fraction ≤ 35% (SLVD group) and those with ejection fraction > 35% (non-SLVD group). RESULTS: We assessed the data of 200 patients [SLVD group, 36 (18%); non-SLVD group, 164 (82%)]). Brain natriuretic peptide level and cardiac resynchronization therapy rate were higher in the SLVD group than in the non-SLVD group. There were no significant between-group differences in major complications and clinical success rates. Patients with SLVD were more likely to require additional hemodynamic support, such as catecholamine infusion, temporary atrium-ventricle sequential pacing, and temporary cardiac resynchronization therapy pacing (27.8% vs 1.2%; P < .001). The survival rate was not significantly different between the groups at 30 days and 1 year after TLE (SLVD vs non-SLVD: 30 days: 97.2% vs 99.4%; P = .215; 1 year: 80.6% vs 91.5%; P = .053). Multivariate Cox regression analysis revealed log brain natriuretic peptide and serum hemoglobin levels as predictors for 1-year mortality. CONCLUSION: The prognosis after TLE was comparable between patients with and without SLVD. However, additional hemodynamic support was often necessary for patients with SLVD.

DOI： 10.1016/j.hrthm.2021.01.026

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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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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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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：English   Publisher：(一社)日本循環器学会  

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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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DOI： 10.1016/j.echo.2020.08.001

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PURPOSE: High-risk patients can be identified by preprocedural computed tomography (CT) before lead extraction. However, CT evaluation may be difficult especially for lead tip identification due to artifacts in the leads. Selective right ventriculography (RVG) may enable preprocedural evaluation of lead perforation. We investigated the efficacy of RVG for identifying right ventricular (RV) lead perforation compared with CT in patients who underwent lead extraction. METHODS: Ninety-five consecutive patients who were examined by thin-section non-ECG-gated multidetector CT and RVG before lead extraction were investigated retrospectively. Newly recognized pericardial effusion after lead extraction was used as a reference standard for lead perforation. We analyzed the prevalence of RV lead perforation diagnosed by each method. The difference in the detection rates of lead perforation by RVG and CT was evaluated. RESULTS: Of the 115 RV leads in the 95 patients, lead perforation was diagnosed for 35 leads using CT, but the leads for 29 (83%) of those 35 leads diagnosed as lead perforation by CT were shown to be within the right ventricle by RVG. Three patients with 5 leads could not be evaluated by CT due to motion artifacts. The diagnostic accuracies of RVG and CT were significantly different (p < 0.001). There was no complication of pericardial effusion caused by RV lead extraction. CONCLUSION: RVG for identification of RV lead perforation leads to fewer false-positives compared to non-ECG-gated CT. However, even in cases in which lead perforation is diagnosed, most leads may be safely extracted by transvenous lead extraction.

DOI： 10.1371/journal.pone.0245502

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Background Sinus tachycardia during exercise attenuates ST-segment elevation in patients with Brugada syndrome, whereas ST-segment augmentation after an exercise test is a high-risk sign. Some patients have premature ventricular contractions (PVCs) related to exercise, but the significance of exercise-related PVCs in patients with Brugada syndrome is still unknown. The objective of this study was to determine the significance of exercise-related PVCs for predicting occurrence of ventricular fibrillation (VF) in patients with Brugada syndrome. Methods and Results The subjects were 307 patients with Brugada syndrome who performed a treadmill exercise test. We evaluated the occurrence of PVCs at rest, during exercise and at the peak of exercise, and during recovery after exercise (0-5 minutes). We followed the patients for 92±68 months and evaluated the occurrence of VF. PVCs occurred in 82 patients (27%) at the time of treadmill exercise test: PVCs appeared at rest in 14 patients (4%), during exercise in 60 patients (20%), immediately after exercise (0-1.5 minutes) in 28 patients (9%), early after exercise (1.5-3 minutes) in 18 patients (6%), and late after exercise (3-5 minutes) in 12 patients (4%). Thirty patients experienced VF during follow-up. Multivariable analysis including symptoms, spontaneous type 1 ECG, and PVCs in the early recovery phase showed that these factors were independently associated with VF events during follow-up. Conclusions PVCs early after an exercise test are associated with future occurrence of VF events. Rebound of vagal nerve activity at the early recovery phase would promote ST-segment augmentation and PVCs in high-risk patients with Brugada syndrome.

DOI： 10.1161/JAHA.120.016907

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

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

DOI： 10.1016/j.jjcc.2020.01.008

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

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AIMS: To establish the indication for programmed ventricular stimulation (PVS) for asymptomatic patients with Brugada syndrome (BrS), we evaluated the prognostic significance of PVS based on abnormal electrocardiogram (ECG) markers. METHODS AND RESULTS: One hundred and twenty-five asymptomatic patients with BrS were included. We performed PVS at two sites of the right ventricle with up to three extrastimuli [two pacing cycle lengths and minimum coupling interval (MCI) of 180 ms]. We followed the patients for 133 months and evaluated ventricular fibrillation (VF) events. Fragmented QRS (fQRS) and Tpeak-Tend (Tpe) interval were evaluated as ECG markers for identifying high-risk patients. Fragmented QRS and long Tpe interval (≥100 ms) were observed in 66 and 37 patients, respectively. Ventricular fibrillation was induced by PVS in 60 patients. During follow-up, 10 patients experienced VF events. Fragmented QRS, long Tpe interval, and PVS-induced VF with an MCI of 180 ms or up to two extrastimuli were associated with future VF events (fQRS: P = 0.015, Tpe ≥ 100 ms: P = 0.038, VF induction: P < 0.001). However, PVS-induced VF with an MCI of 200 ms was less specific (P = 0.049). The frequencies of ventricular tachyarrhythmia events during follow-up were 0%/year with no ECG markers and 0.1%/year with no VF induction. The existence of two ECG factors with induced VF was strongly associated with future VF events (event rate: 4.4%/year, P < 0.001), and the existence of one ECG factor with induced VF was also associated (event rate: 1.3%/year, P = 0.011). CONCLUSION: We propose PVS with a strict protocol for asymptomatic patients with fQRS and/or long Tpe interval to identify high-risk patients.

DOI： 10.1093/europace/euaa003

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BACKGROUND: The aim of this study was to evaluate the efficacy and safety of transdermal β-blocker patches, which offer stable blood concentration and easy availability during operation, for prevention of perioperative myocardial injury (PMI) in high-risk patients.Methods and Results:In this randomized controlled trial, patients aged >60 years with hypertension and high revised cardiac risk index (≥2) undergoing non-cardiac surgery were randomly assigned to a bisoprolol patch or control group. Primary efficacy outcome was incidence of PMI, defined as postoperative high-sensitivity cardiac troponin T (hs-cTnT) >0.014ng/mL and relative hs-cTnT change ≥20%. Secondary efficacy outcomes were number of cardiovascular events and 30-day mortality. From November 2014 to February 2019, 240 patients from 5 hospitals were enrolled in this study. The incidence of PMI was 35.7% in the bisoprolol patch group and 44.5% in the control group (P=0.18). Incidence of major adverse cardiac events including non-critical myocardial infarction, strokes, decompensated heart failure and tachyarrhythmia was similar between the 2 groups. Tachyarrhythmia tended to be higher in the control group. There were no significant differences in safety outcomes including significant hypotension and bradycardia requiring any treatment between the 2 groups. CONCLUSIONS: Bisoprolol patches do not influence the incidence of PMI and cardiovascular events in high-risk patients undergoing non-cardiac surgery, but perioperative use of these patches is safe.

DOI： 10.1253/circj.CJ-19-0871

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BACKGROUND: Multiple spikes in the QRS complex (fragmented QRS [fQRS]) on 12-lead electrocardiography have been associated with ventricular arrhythmic events (VAEs) in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to assess the association between new appearances of fQRS and cardiac events in patients with HCM.Methods and Results:The association between baseline fQRS and cardiac events, namely VAEs, heart failure-related hospitalization, and all-cause death, was evaluated retrospectively in 146 HCM patients (46 patients with fQRS, 100 without fQRS). The median follow-up was 5.3 years. Cardiac events occurred in 29 patients with baseline fQRS and 32 patients without baseline fQRS (63% vs. 32%; P<0.001). VAEs occurred in a significantly larger percentage of patients with than without baseline fQRS (54% vs. 23%, respectively; P<0.001). Of the 100 patients without baseline fQRS, 33 had a new appearance of fQRS during the 4.6-year follow-up, whereas 67 did not. VAEs occurred more frequently in the 33 patients with the appearance of fQRS than in those without (42% vs. 13%, respectively; P=0.001). Multivariable analysis showed that the new appearance of fQRS documented before VAEs was associated with VAEs (hazard ratio 4.29, 95% confidence interval 1.81-10.2; P=0.001). CONCLUSIONS: The new appearance of fQRS was associated with an increased risk of VAEs in HCM patients.

DOI： 10.1253/circj.CJ-19-0968

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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   Publisher：日本成人先天性心疾患学会  

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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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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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In order to prevent ischemic stroke, it is important to identify and treat patients with atrial fibrillation (AF) who do not consult a doctor in a medical institution. The aim of this study was to determine the consultation rate at medical institutions for patients with AF in group medical examinations conducted in a city in western Japan. Of 6101 examinees of group medical examinations (40 years of age or older) conducted in Ibara City, Okayama Prefecture, Japan, from 2012 to 2014, 4338 participants (71.1%) who were evaluated by electrocardiogram (ECG) gave written informed consent and responded to surveys in the form of questionnaires through a personal interview conducted by nurses were included in the Ibara-AF study. A cumulative total of 82 subjects were diagnosed as having AF by ECG (prevalence of AF = 1.89%), and 51 individuals had AF during the three-year period.15 (29.4%) of the 51 patients with AF did not regularly visit medical institutions. Among them, 46.7% (n = 7) and 53.3% (n = 8) of the patients were symptomatic and asymptomatic, respectively, and 73.3% of the patients had a CHADS2 score of more than one point. There were no significant differences in patients' characteristics between regular and non-regular visit groups. In conclusion, about one-third of the patients with AF did not regularly see a doctor in a medical institution and most of them had a CHADS2 score of more than one point in a Japanese rural area. Educating the public about the risks of AF is required.

DOI： 10.1536/ihj.19-062

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There are three critical pathways for the pathogenesis and progression of pulmonary arterial hypertension (PAH): the prostacyclin (prostaglandin I2) (PGI2), nitric oxide (NO), and endothelin pathways. The current approved drugs targeting these three pathways, including prostacyclin (PGI2), phosphodiesterase type-5 (PDE5) inhibitors, and endothelin receptor antagonists (ERAs), have been shown to be effective, however, PAH remains a severe clinical condition and the long-term survival of patients with PAH is still suboptimal. The full therapeutic abilities of available drugs are reduced by medication, patient non-compliance, and side effects. Nanoparticles are expected to address these problems by providing a novel drug delivery approach for the treatment of PAH. Drug-loaded nanoparticles for local delivery can optimize the efficacy and minimize the adverse effects of drugs. Prostacyclin (PGI2) analogue, PDE5 inhibitors, ERA, pitavastatin, imatinib, rapamycin, fasudil, and oligonucleotides-loaded nanoparticles have been reported to be effective in animal PAH models and in vitro studies. However, the efficacy and safety of nanoparticle mediated-drug delivery systems for PAH treatment in humans are unknown and further clinical studies are required to clarify these points.

DOI： 10.3390/ijms20235885

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

<title>Abstract</title>
<sec>
<title>Aims</title>
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.


</sec>
<sec>
<title>Methods and results</title>
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].


</sec>
<sec>
<title>Conclusion</title>
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.


</sec>

DOI： 10.1093/europace/euz207

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Objective Remote monitoring (RM) of cardiac implantable electric devices (CIEDs) has been advocated as a healthcare standard. However, expert consensus statements suggest that all patients require annual face-to-face follow-up consultations at outpatient clinics even if RM reveals no episodes. The objective of this study was to determine the critical event rate after CIED implantation through RM. Methods This multicenter, retrospective, cohort study evaluated patients with pacemakers (PMs), implantable cardioverter defibrillators (ICDs), or cardiac resynchronization therapy defibrillator (CRT-Ds) and analyzed whether or not the data drawn from RM included abnormal or critical events. Patients A total of 1,849 CIED patients in 12 hospitals who were followed up by the RM center in Okayama University Hospital were included in this study. Results During the mean follow-up period of 774.9 days, 16,560 transmissions were analyzed, of which 11,040 (66.7%) were abnormal events and only 676 (4.1%) were critical events. The critical event rate in the PM group was significantly lower than that in the ICD or CRT-D groups (0.9% vs. 5.0% or 5.9%, p<0.001). A multivariate analysis revealed that ICD, CRT-D, and a low ejection fraction were independently associated with critical events. In patients with ICD, the independent risk factors for a critical event were old age, low ejection fraction, Brugada syndrome, dilated phase hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. Conclusion Although abnormal events were observed in two-thirds of the transmitted RM data, the critical event rate was <1% in patients with a PM, which was lower in comparison to the rates in patients with ICDs or CRT-Ds. A low ejection fraction was an independent predictor of critical events.

DOI： 10.2169/internalmedicine.1905-18

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

DOI： 10.1002/joa3.12164

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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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Recent large clinical trials failed to show clear benefits of percutaneous transluminal renal angioplasty (PTRA) as compared with medical therapy on patients with renal artery stenosis. It was also reported that proteinuria is an adverse prognostic factor after PTRA, and PTRA is less effective in patients with overt proteinuria. From the renoprotective point of view, to reduce proteinuria after PTRA is an important therapeutic goal in patients with renal artery stenosis with overt proteinuria. We hereby describe two patients successfully treated by combination therapy with PTRA and administration of angiotensin-converting enzyme (ACE) inhibitor for bilateral renal artery disease with overt proteinuria.

DOI： 10.2169/internalmedicine.2076-18

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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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PURPOSE OF REVIEW: To summarize the current status of coronary computed tomography angiography (CTA) in the assessment of coronary plaques and discuss the ability of serial coronary CTA to quantitatively measure changes in the plaque burden in response to lipid-lowering therapy. RECENT FINDINGS: Recent advances in coronary CTA have allowed identification of high-risk coronary features in acute coronary syndrome and measurement of changes in the coronary plaque burden with good reproducibility. Statin therapy may delay plaque progression and change some plaque features. However, the clinical relevance of quantitative changes in coronary plaques and the optimal methods to reduce the plaque burden remain unclear. Despite guideline-directed lipid-lowering therapy, adverse events still occur in substantial numbers of patients receiving statins. Coronary CTA is noninvasive and has high diagnostic performance in patients with coronary artery disease, making change in the plaque burden an applicable biomarker for individualized assessment of future risk.

DOI： 10.1007/s11886-019-1170-4

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Atrial fibrillation (AF) frequently coexists with cardiovascular disease (CAD) in a clinical setting. However, the optimum therapy for AF patients who have concomitant CAD is unclear. We retrospectively examined the efficacy and safety of radiofrequency catheter ablation (RFCA) prior to percutaneous coronary intervention (PCI) in patients with AF who had concomitant stable CAD. Between January 2014 and December 2015, a total 264 patients (179 men; mean age, 65.5 ± 10.1 years) who were referred to undergo a first RFCA procedure were reviewed in this study. Of the 264 patients, 41 (15.5%) had stable CAD detected by multi-detector computed tomography before RFCA. Thirty-seven patients who had AF with stable CAD were divided into two treatment arms: (1) RFCA prior to PCI (n = 13) and (2) PCI prior to RFCA (n = 24) [four patients excluded because of left main coronary artery disease (LMCA) or triple vessel disease (TVD)]. The median follow-up was 14 (IQR 8-19) months. There was no significant difference in AF recurrence rate after the procedure between the RFCA first group and PCI first group (P = 0.515). No symptomatic cardiovascular events occurred the during follow-up period. The PCI first group had a significantly longer duration of triple therapy (188.5 ± 167 days vs 5.6 ± 24.5 days, P = 0.01) and all of the four bleeding events occurred during triple therapy (P = 0.01). The results of this single-center pilot study suggested that prior RFCA in patients with AF coexisting with CAD could have fewer serious bleeding events than prior PCI.

DOI： 10.1007/s00380-018-1280-8

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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)日本集中治療医学会  

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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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BACKGROUND: Various risk stratifications in asymptomatic patients with Brugada syndrome (BrS) have been proposed, but the electrophysiological change that promotes ventricular fibrillation (VF) is still unknown. OBJECTIVE: The aim of this study was to clarify the changes in electrocardiographic (ECG) markers at the onset of VF from ECGs recorded when patients were still asymptomatic. METHODS: The subjects of this study included 14 patients with VF and 48 consecutive asymptomatic patients with BrS. We compared ECGs before the initial VF events (>6 months; early phase) with ECGs at the initial VF events (late phase). In asymptomatic patients, we evaluated ECGs at 2 time points with an interval of >6 months. We evaluated various ECG markers including type 1 ECG and fragmented QRS (fQRS; multiple spikes within the QRS complex). RESULTS: ECG parameters of the early and late phases were not different except for decreased ST voltage and low incidence of type 1 ECG in asymptomatic patients. There were no differences in ECG parameters of the early phase between patients with VF and asymptomatic patients. In patients with VF, ECGs at the late phase had longer QRS intervals and intervals between the peak and the end of the T wave and more frequent type 1 ECG and fQRS than did ECGs at the early phase. Those changes were associated with initial VF events (QRS widening: odds ratio [OR] 11.5, P < .01; interval between the peak and the end of the T wave: OR 11.6, P < .01; fQRS: odds ratio 15.3, P < .01; type 1 ECG: OR 6.6, P < .05). CONCLUSION: QRS and ST-T wave abnormalities developed in association with the initial VF events. Aggravation of the conduction disturbance in addition to BrS-ECG promotes VF.

DOI： 10.1016/j.hrthm.2018.06.035

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

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OBJECTIVES: The principal objective was to perform an initial test of the Shanghai Brugada Scoring System. Diagnosis of probable and/or definite Brugada syndrome (BrS), possible BrS, and nondiagnostic outcomes were assigned scores of ≥3.5, 2 to 3, and <2 points, respectively. The proposed score system was based on the available published reports and on weighted coefficients derived from limited datasets, with the understanding that these recommendations would need to undergo continuing validation. BACKGROUND: The 2016 HRS/EHRA/APHRS/SOLAECE J-Wave Syndrome Consensus Report proposed a scoring system for diagnosis of BrS that takes into account electrocardiographic recordings, genetic results, clinical characteristics, and family history. METHODS: The patient population consisted of 393 patients evaluated at our hospital for BrS (271 asymptomatic, 99 with syncope, and 23 with ventricular fibrillation [VF]) between 1996 and 2016. Subjects were classified into 4 groups: group A with a score of ≤3.0 points (n = 45); group B with a score of 3.5 points (n = 186); group C with a score of 4.0 to 5.0 points (n = 81); and group D with a score of ≥5.5 points (n = 81). RESULTS: A total of 348 (88%) patients had probable and/or definite BrS, and 81 (20%) had a score ≥5.5. During a follow-up of 97.3 months (range: 39.7 to 142.1 months), 43 patients experienced VF. Significant differences were seen among the 4 groups (p = 0.01). A malignant arrhythmic event did not occur in any patient with possible or nondiagnostic BrS. CONCLUSIONS: This study provided validation for the use of the Shanghai Score System for the diagnosis and risk stratification of patients with BrS.

DOI： 10.1016/j.jacep.2018.02.009

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BACKGROUND: A drug provocation test using a sodium channel blocker (SCB) can unmask a type 1 ECG pattern in patients with Brugada syndrome. However, the prognostic value of the results of an SCB challenge is limited in patients with non-type 1 ECG. We investigated the associations of future risk for ventricular fibrillation with SCB-induced ECG changes and ventricular tachyarrhythmias (VTAs). METHODS AND RESULTS: We administered intravenous pilsicainide to 245 consecutive patients with Brugada syndrome (181 patients with spontaneous type 1 ECG, 64 patients with non-type 1 ECG). ECG parameters before and after the test and occurrence of drug-induced VTAs were evaluated. During a mean follow-up period of 113±57 months, fatal VTA events occurred in 31 patients (sudden death: n=3, ventricular tachycardia/ventricular fibrillation: n=28). Symptomatic patients and spontaneous type 1 ECG were associated with future fatal arrhythmic events. Univariable analysis of ECG parameters after the test showed that long PQ and QRS intervals, high ST level, and SCB-induced VTAs were associated with later VTA events during follow-up. Multivariable analysis showed that symptomatic patients, high ST level (V1) ≥0.3 mV after the test, and SCB-induced VTAs were independent predictors for future fatal arrhythmic events (hazard ratios: 3.28, 2.80, and 3.62, 95% confidence intervals: 1.54-7.47, 1.32-6.35, and 1.64-7.75, respectively; P<0.05). CONCLUSIONS: SCB-induced VTAs and ST-segment augmentation are associated with an increased risk of the development of ventricular tachycardia/ventricular fibrillation events during follow-up in patients with Brugada syndrome.

DOI： 10.1161/JAHA.118.008617

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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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BACKGROUND: Remote monitoring (RM) has been advocated as the new standard of care for patients with cardiovascular implantable electronic devices (CIEDs). RM has allowed the early detection of adverse clinical events, such as arrhythmia, lead failure, and battery depletion. However, lead failure was often identified only by arrhythmic events, but not impedance abnormalities. OBJECTIVE: To compare the usefulness of arrhythmic events with conventional impedance abnormalities for identifying lead failure in CIED patients followed by RM. METHODS: CIED patients in 12 hospitals have been followed by the RM center in Okayama University Hospital. All transmitted data have been analyzed and summarized. RESULTS: From April 2009 to March 2016, 1,873 patients have been followed by the RM center. During the mean follow-up period of 775 days, 42 lead failure events (atrial lead 22, right ventricular pacemaker lead 5, implantable cardioverter defibrillator [ICD] lead 15) were detected. The proportion of lead failures detected only by arrhythmic events, which were not detected by conventional impedance abnormalities, was significantly higher than that detected by impedance abnormalities (arrhythmic event 76.2%, 95% CI: 60.5-87.9%; impedance abnormalities 23.8%, 95% CI: 12.1-39.5%). Twenty-seven events (64.7%) were detected without any alert. Of 15 patients with ICD lead failure, none has experienced inappropriate therapy. CONCLUSIONS: RM can detect lead failure earlier, before clinical adverse events. However, CIEDs often diagnose lead failure as just arrhythmic events without any warning. Thus, to detect lead failure earlier, careful human analysis of arrhythmic events is useful.

DOI： 10.1111/jce.13399

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BACKGROUND: High-sensitivity cardiac troponin T (hs-cTnT) is useful for detecting myocardial injury and is expected to become a prognostic marker in patients undergoing non-cardiac surgery. The aim of this pilot study evaluating the efficacy of β-blocker therapy in a perioperative setting (MAMACARI study) was to assess perioperative myocardial injury (PMI) in elderly patients with preserved ejection fraction (EF) undergoing non-cardiac surgery.Methods and Results:In this prospective observational cohort study of 151 consecutive patients with preserved EF and aged >60 years who underwent non-cardiac surgery, serum levels of hs-cTnT were measured before and on postoperative days 1 and 3 after surgery. PMI was defined as postoperative hs-cTnT >0.014 ng/mL and relative hs-cTnT change ≥20%. A total of 36 (23.8%) of the patients were diagnosed as having PMI. The incidence of a composite of cardiovascular events within 30 days after surgery, including myocardial infarction, stroke, worsening heart failure, atrial fibrillation and pulmonary embolism, was significantly higher in patients with PMI than in patients without PMI (odds ratio (OR) 9.25, P<0.001, 95% confidence interval (CI) 2.65-32.3). Multivariate analysis revealed that left ventricular diastolic dysfunction defined by echocardiography was independently associated with PMI (OR: 3.029, 95% CI: 1.341-6.84, P=0.008). CONCLUSIONS: PMI is frequently observed in elderly patients undergoing non-cardiac surgery. Diastolic dysfunction is an independent predictor of PMI.

DOI： 10.1253/circj.CJ-17-0747

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

BACKGROUND: In Brugada syndrome (BrS), it has been reported that delayed activation in the RV is related to the development of type-1 ECG, which is more critical than type-2. On the other hand, the coexistence of complete right bundle-branch block (CRBBB), which also causes delayed activation in the RV, sometimes makes typical BrS ECG misleading. We hypothesized that premature stimulation of the RV can unmask the influence of delayed activation in the RV and convert the morphology of ECG in BrS patients. METHODS AND RESULTS: In 35 BrS patients with type-1 ECG including 8 patients with concomitant CRBBB and 6 control subjects with CRBBB, progressively premature single stimulations were delivered from the RV apex on electrophysiological study. Then we evaluated QRS morphology of fusion beats created by single premature stimulation in each patient. In 29 (83%) of 35 of the BrS patients, conversion from type-1 to type-2 ECG was observed during the process of single premature stimulation. Additionally, in all 8 BrS patients with concomitant CRBBB, type-1 or type-2 BrS ECG was revealed by premature stimulation with relief of CRBBB. These findings were not observed in any of the control subjects with CRBBB. CONCLUSION: Single premature stimulation of the RV converts ECG from type-1 to type-2 in most BrS cases and unmasks type-1 ECG in all BrS cases with CRBBB. Our results could suggest that type-1 ECG is associated with delayed activation of the RV compared with type-2 ECG.

DOI： 10.1111/jce.13336

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INTRODUCTION: New onset of ventricular fibrillation (VF) in asymptomatic patients with Brugada-type ECG is not frequent, but it cannot be negligible. Risk markers for predicting VF are usually based on results of analysis in symptomatic patients, and they have not been determined for asymptomatic patients. We analyzed ECG markers in patients with Brugada syndrome to differentiate the risk factors for VF in both symptomatic and asymptomatic patients. METHODS: The subjects were 471 patients with Brugada syndrome and we divided the subjects into two groups: Asymptomatic group (n = 326) and Symptomatic group (syncope: n = 122, VF: n = 23). We analyzed the following ECG markers: RR, PQ, QRS, QT and Tpeak-Tend (Tpe) intervals, ST level, atrial fibrillation (AF), atrioventricular block, spontaneous type 1 ECG, early repolarization (ER) and fragmented QRS (fQRS). RESULTS: During follow-up (91 ± 64 months), 41 patients experienced VF (Asymptomatic: n = 10, Symptomatic: n = 31). Univariable analysis showed that spontaneous type 1 ECG, Tpe interval (≥95 milliseconds), high ST level (≥0.52 mV) and fQRS were common predictors for VF in both the Asymptomatic and Symptomatic groups. In addition to the common risk factors, wide QRS (≥107 milliseconds), long QT interval (≥420 milliseconds), ER and AF were predictors for VF in Symptomatic group. Multivariable analysis of the Symptomatic group showed fQRS, Tpe and ER were independent predictors of prognosis. CONCLUSIONS: fQRS and Tpe interval are common risk factors for VF in both asymptomatic and symptomatic patients, whereas ER is a predictor for recurrent VF.

DOI： 10.1111/jce.13349

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BACKGROUND: Pulmonary vascular remodeling of pulmonary arterial hypertension (PAH) is characterized by an inappropriate increase of vascular cells. The receptor for advanced glycation end products (RAGE) is a type I single-pass transmembrane protein belonging to the immunoglobulin superfamily and is involved in a broad range of hyperproliferative diseases. RAGE is also implicated in the etiology of PAH and is overexpressed in pulmonary artery smooth muscle cells (PASMCs) in patients with PAH. We examined the role of RAGE in the inappropriate increase of PASMCs in patients with PAH. METHODS AND RESULTS: PASMCs were obtained from 12 patients with PAH including 9 patients with idiopathic PAH (IPAH) and 3 patients with heritable PAH (HPAH) (2 patients with BMPR2 mutation and one patient with SMAD9 mutation) who underwent lung transplantation. Western blot analysis and immunofluorescence staining revealed that RAGE and S100A8 and A9, ligands of RAGE, were overexpressed in IPAH and HPAH-PASMCs in the absence of any external growth stimulus. PDGF-BB (10 ng/mL) up-regulated the expression of RAGE in IPAH and HPAH-PASMCs. PAH-PASMCs are hyperplastic in the absence of any external growth stimulus as assessed by 3H-thymidine incorporation. This result indicates overgrowth characterized by continued growth under a condition of no growth stimulation in PAH-PASMCs. PDGF-BB stimulation caused a higher growth rate of PAH-PASMCs than that of non-PAH-PASMCs. AS-1, an inhibitor of TIR domain-mediated RAGE signaling, significantly inhibited overgrowth characterized by continued growth under a condition of no growth stimulation in IPAH and HPAH-PASMCs (P<0.0001). Furthermore, AS-1 significantly inhibited PDGF-stimulated proliferation of IPAH and HPAH-PASMCs (P<0.0001). CONCLUSIONS: RAGE plays a crucial role in the inappropriate increase of PAH-PASMCs. Inhibition of RAGE signaling may be a new therapeutic strategy for PAH.

DOI： 10.1371/journal.pone.0203046

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BACKGROUND: The eligibility of patients with Brugada syndrome (BrS) for implantation of a subcutaneous implantable cardioverter defibrillator (S-ICD) is not well known. This study aimed to clarify the eligibility of BrS patients for S-ICD using electrocardiography (ECG) at rest and during exercise testing. We also analyzed factors associated with ineligibility for S-ICD from standard 12-lead ECG at rest. METHODS: We enrolled 110 consecutive BrS patients who visited Okayama university hospital from December 2015 to December 2016. All patients were assessed for S-ICD eligibility, which required one lead to satisfy the S-ICD screening template. We assessed standard 12-lead ECG parameters in all participants. Of those who passed S-ICD screening, 45 patients were assessed for S-ICD eligibility during treadmill stress test. RESULTS: Mean age of study patients was 54 ± 13 years and 108 (98%) were men. In total, 89 patients (81%) satisfied S-ICD indications at rest. Existence of complete right bundle branch block (CRBBB) on standard 12-lead ECG was a significant predictor of ineligibility for S-ICD (odds ratio, 5.00; P = 0.03; 95%CI, 1.14-21.98). Of the 45 patients who underwent treadmill stress testing, 11 patients (24%) showed ineligibility for S-ICD during the test. CONCLUSION: CRBBB was a predictor of ineligibility for S-ICD in patients with BrS. Sinus tachycardia changes ECG morphology in some patients and stress testing should be considered before S-ICD implantation.

DOI： 10.1111/jce.13315

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OBJECTIVES: This study aimed to assess the fate of tricuspid regurgitation (TR) after transcatheter atrial septal defect (ASD) closure. BACKGROUND: Although TR frequently occurs in patients with ASD, the change in TR during long-term follow-up after ASD closure remains unknown. METHODS: A total of 419 adult patients who underwent transcatheter ASD closure were enrolled. TR severity was graded by TR jet area on echocardiography. 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. 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.

DOI： 10.1016/j.jcin.2017.06.022

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BACKGROUND: An early failure of the Biotronik Linox S/SD implantable cardioverter-defibrillator (ICD) lead has been reported. We have also experienced several cases with early failure of Linox leads. OBJECTIVE: Our aim was to assess the longevity of Linox S/SD (Biotronik, Berlin, Germany) compared with Sprint Fidelis (Medtronic, Minneapolis, MN), Sprint Quattro (Medtronic), and Endotak Reliance (Boston Scientific, Natick, Massachusetts) leads. METHODS: We retrospectively reviewed patients who had undergone implantation of Linox S/SD (n = 90), Sprint Fidelis (n = 37), Sprint Quattro (n = 27), or Endotak Reliance (n = 50) leads between June 2000 and December 2013 at our hospital. Variables associated with lead failure were assessed by the Kaplan-Meier method and Cox survival modeling. RESULTS: Failure rates of Linox, Sprint Fidelis, and Endotak leads were 3.2%/year (7-year survival rate, 81.0%), 3.4%/year (7-year survival rate, 77.2%), and 0.61%/year (7-year survival rate, 95.8%), respectively. No lead failure was found with Sprint Quattro leads. The survival probability of Linox leads was significantly lower than that of Endotak leads (P = .049) and comparable to that of Sprint Fidelis leads (P = .69). In univariate analysis, age was the only predictor of Linox lead failure. Patients <58 years old were at significantly increased risk of lead failure compared with patients ≥58 years old (hazard ratio, 9.0; 95% confidence interval, 1.13-71.3; P = .037). CONCLUSION: In our single-center experience, the survival rate of Linox leads was unacceptably low. The only predictor of Linox lead failure was age at implantation. This is the first description of a lower survival rate for Linox leads in an Asian population.

DOI： 10.1016/j.hrthm.2017.05.020

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

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BACKGROUND: The subcutaneous implantable cardioverter-defibrillator (S-ICD) is recognized as a viable alternative to the transvenous ICD. The safety and efficacy of this device has been demonstrated in Western countries, but studies with S-ICD implantation in Japanese patients have not been reported. METHODS AND RESULTS: Twelve patients received an S-ICD implant in our institute between February and September 2016. All S-ICDs were successfully implanted without complications. One appropriate and one inappropriate therapy was identified. CONCLUSIONS: S-ICD implantation appears to provide a viable alternative to transvenous ICD implantation for some Japanese patients. However, we should perform careful follow-up of patients to eliminate inappropriate therapy.

DOI： 10.1016/j.joa.2017.02.004

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

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BACKGROUND: In order to minimize inappropriate shocks of subcutaneous implantable cardioverter-defibrillators (S-ICD), it is important to recognize who is suitable for S-ICD indication. This study aimed to clarify what types of cardiac disease are likely to fulfill the S-ICD screening criteria and ineligible factors for S-ICD in the standard 12-lead electrocardiogram (ECG). METHODS: A total of 348 patients with heart disease were enrolled. They were assessed by supine and standing ECG recording to simulate the 3 S-ICD sensing vectors and standard 12-lead ECG, simultaneously. Clinical and ECG characteristics were analyzed to compare the patients who are eligible and ineligible with S-ICD screening ECG indication. RESULTS: The mean age of study patients was 49±21 years and 244 (70%) were men. Nineteen percent of patients were unsuitable for S-ICD. There was no significant difference in ineligibility for S-ICD among cardiac diseases (p=0.48). Univariate analysis showed complete right bundle branch block (CRBBB), QRS-T discordance in lead II, and QRS-T discordance in 3 leads (I, II, and aVF) were more frequent in patients who were ineligible for S-ICD than in the eligible group. Multivariate regression analysis showed CRBBB and QRS-T discordance in 3 leads were independent predictors for ineligibility of S-ICD. CONCLUSION: There are no differences in eligibility of S-ICD among types of cardiac diseases. CRBBB and QRS-T discordance were independent predictors for ineligibility.

DOI： 10.1016/j.jjcc.2016.11.014

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DOI： 10.1016/j.hrcr.2017.05.001

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A 7-year-old boy presented at our hospital with syncope. At birth, electrocardiography had shown a long QT interval with torsade de pointes (TdP). Congenital long QT syndrome (LQTS) had been diagnosed by genetic testing, and was successfully controlled with oral propranolol. At age 7, TdP had recurred with syncope. Electrocardiography revealed a prominent long QT interval with T-wave alternans. The propranolol dose was increased, but TdP remained uncontrolled. A cardioverter-defibrillator (ICD) was implanted epicardially, and TdP completely resolved with atrial pacing. We report this rare case of ICD implantation in a child with LQTS.

DOI： 10.1016/j.joa.2016.10.561

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T wave oversensing (TWOS) is a common cause of inappropriate implantable cardioverter-defibrillator (ICD) therapies. Various algorithms to avoid inappropriate ICD therapy are available; however, they are not helpful to avoid TWOS. Although the reproduction of TWOS is useful to resolve the problem of TWOS, it is sometimes difficult to reproduce TWOS. We report two cases of inappropriate ICD therapy due to TWOS, which were induced only by body twisting. We can successfully manage the device based on the evidence of reproduced TWOS.

DOI： 10.1016/j.joa.2016.06.003

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BACKGROUND: Fragmented QRS complexes (fQRS) in the right precordial leads are associated with occurrence of ventricular fibrillation (VF) in Brugada syndrome. Recently, epicardial mapping has revealed abnormal electrograms at the right ventricular (RV) outflow tract and inferior region of the right ventricle. fQRS may reflect the extent of the area of abnormal potentials, but whether the distribution of fQRS has prognostic value is not known. METHODS AND RESULTS: We evaluated the existence of fQRS in 456 patients with Brugada syndrome, including 117 patients with syncope and 23 patients with VF. The region of fQRS was defined as inferior (II, III, and aVF), lateral (I, aVL, and V5 and V6), anterior (V3 and V4), RV (V1 and V2), and RV outflow tract (V1 and V2 at the third intercostal space). fQRS were present in 229 patients (RV outflow tract in 175, inferior in 135, RV in 90, and lateral in 16 patients). During follow-up (mean 91 months), 39 patients experienced VF. In univariable analyses, fQRS in any distribution and fQRS in each region excluding the RV were associated with VF. Multivariable analysis showed that fQRS in the inferior (hazard ratio, 3.9; confidence interval, 1.9-8.5), lateral (hazard ratio, 3.5; confidence interval, 1.2-8.2), and RV outflow tract (hazard ratio, 2.5; confidence interval, 1.2-5.6) were associated with VF events. The presence of multiple regions of fQRS was associated with worse prognosis. CONCLUSIONS: The distribution of fQRS is associated with prognosis in Brugada syndrome, further supporting the association of fQRS and arrhythmia substrate.

DOI： 10.1161/CIRCEP.116.004765

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BACKGROUND: Patients with pulmonary arterial hypertension (PAH) are currently treated with combination therapy of PAH-targeted drugs. Reverse right ventricular (RV) remodeling after lung transplantation (LTx) in patients with end-stage PAH despite combination therapy of PAH-targeted drugs has not been fully elucidated.Methods and Results:A total of 136 patients, including 32 with PAH, underwent LTx from 1998 to 2014. We enrolled 12 consecutive patients with PAH treated with combination therapy of PAH-targeted drugs who underwent LTx and retrospectively analyzed the temporal and serial changes in hemodynamics and echocardiography before LTx and at 3 and 12 months after LTx. Before LTx, the RV was markedly dilated with substantially reduced RV fractional area change (RVFAC). At 3 months after LTx, pulmonary artery pressure, pulmonary vascular resistance and RV stroke work index were significantly decreased, while left ventricular stroke work index was increased. RV size assessed by echocardiography also significantly decreased and RVFAC improved. At 12 months after LTx, RVFAC was further increased and RV wall thickness was decreased significantly. CONCLUSIONS: Although severe RV dysfunction and dilation were observed in patients with end-stage PAH despite combination therapy of PAH-targeted drugs, RV function and morphology were improved after reduction of RV pressure load by LTx.

DOI： 10.1253/circj.CJ-16-0838

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OBJECTIVE: This 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. BACKGROUND: Although 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. METHODS: Two 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'] <5.0 cm/s), mild (5.0≤ e' <8.0 cm/s), and normal (e' ≥ 8.0 cm/s). Changes in plasma B-type natriuretic peptide (BNP) levels were evaluated. RESULTS: No 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 (126 ± 181 to 131 ± 148 pg/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 ± 131 pg/ml, P = 0.724). CONCLUSIONS: Our 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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Background: Brugada syndrome (BrS) is characterized by J-point or ST-segment elevation on electrocardiograms (ECGs) and increased risk of ventricular fibrillation (VF). In BrS, epicardial depolarization abnormality with delayed potential on the right ventricular outflow tract is reportedly the predominant mechanism underlying VF. Yet VF occurrence is also associated with early repolarization (ER) pattern in the inferolateral ECG leads, which may represent the inferior and/or left lateral ventricular myocardium. The aim of this study was to examine epicardial electrograms recorded directly at the left ventricle (LV) in BrS patients after VF episodes. Methods: In 12 BrS patients who had experienced VF episodes and 17 control subjects, a multipolar catheter was introduced into the left lateral coronary vein for unipolar and bipolar electrogram recordings at the LV epicardium. Both inferior and lateral ER patterns on ECG were observed in three BrS patients and six control subjects. Results: In the epicardium, prominent J waves were detected using unipolar recording, and potentials after the QRS complex were detected using bipolar recording in three of the 12 BrS patients. These three patients also showed both inferior and lateral ER patterns on ECG. Neither prominent J waves nor potentials after the QRS complex were recorded at the endocardium of the LV in any of these three patients; nor were they seen at the epicardium in any of the control subjects. These features were accentuated on pilsicainide administration (n = 2) but diminished on constant atrial pacing (n = 3) and isoproterenol administration (n = 1). The J waves observed through unipolar recording coincided with the potentials after QRS complex observed through bipolar recording and with the inferolateral ER patterns on ECG. Conclusions: We recorded prominent J waves in unipolar electrogram and potentials after QRS complex in bipolar electrogram at the LV epicardium in BrS patients with global ER pattern. The prominent J waves coincided with the potentials after QRS complex and the inferolateral ER pattern on ECG. The characteristics of the inferolateral ER pattern on ECG in these patients primarily represent depolarization feature.

DOI： 10.3389/fphys.2017.00014

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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.

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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 >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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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, 46 ± 22 years) with ostium secundum ASDs measuring ≤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 (<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 < .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 < .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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BACKGROUND: Clinical and experimental studies have shown the existence of an arrhythmogenic substrate in the right ventricular outflow tract (RVOT) in patients with Brugada syndrome (BrS). To evaluate the importance of the RVOT, we evaluated the activation pattern of induced ventricular tachyarrhythmias using body surface mapping (BSM) in patients with BrS. METHODS AND RESULTS: We examined 14 patients with BrS in whom ventricular tachyarrhythmias were induced by programmed electrical stimulation. The 87-lead BSM was recorded during induced ventricular tachyarrhythmias, and an activation map and an isopotential map of QRS complexes every 5 ms were constructed to evaluate the activation pattern of ventricular tachyarrhythmias. BSM during 20 episodes of ventricular tachyarrhythmias induced at the RVOT showed that repetitive excitation was generated at the RVOT and propagated to the inferior RV and left ventricle, and then returned to the RVOT. Polymorphic QRS change during ventricular tachyarrhythmias was associated with migration of the earliest activation site and rotor. BSM during 4 episodes of ventricular fibrillation (VF) showed that the excitation front moved randomly with formation of multiple wavefronts. CONCLUSIONS: Programmed stimulation initiated repetitive firing from the RVOT. Migration and competition of the earliest activation site and rotor and local conduction delay changed the QRS morphology. Degeneration of the reentrant circuit into multiple wavefronts resulted in VF. (Circ J 2016; 80: 1734-1743).

DOI： 10.1253/circj.CJ-16-0124

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

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DOI： 10.1016/j.jcin.2016.03.014

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

BACKGROUND: A therapeutic strategy in patients with atrial septal defect (ASD) and significant pulmonary arterial hypertension (PAH) remains controversial. This study aimed to assess the effect of PAH-specific medications and subsequent transcatheter shunt closure (ie, a treat and repair strategy) in these patients. METHODS AND RESULTS: Among 646 patients with ASD, 22 patients (mean age of 56±20 years) who had PAH [mean pulmonary artery pressure ≥25 mmHg and pulmonary vascular resistance (PVR) ≥3 Wood units] underwent successful transcatheter ASD closure. Prior to the procedure, 8 patients received PAH-specific medications (PHM group) and 14 patients did not (non-PHM group). Initially, the PHM group had higher PVR compared with non-PHM group (9.6±3.8 vs. 4.2±1.0 Wood units, P<0.01). After treatment with PAH-specific medications, PVR in this group decreased to 4.0±0.8 Wood units (P<0.01). No adverse events were observed in either the PHM or non-PHM group during or after the transcatheter procedure. In the PHM group, during a treatment period of 52±48 months, the World Health Organization Functional Classification significantly improved (3.0±0.5 to 2.0±0.0, P<0.01), as well as in the non-PHM group (2.1±0.6 to 1.5±0.5, P<0.01). CONCLUSIONS: Treat and repair strategy provided substantial improvement and no worsening of the WHO-FC, even in patients with ASD and significant PAH. Long-term hemodynamic follow-up is mandatory to evaluate the ultimate efficacy and safety of this new strategy.

DOI： 10.1253/circj.CJ-15-0599

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Although the volume overload of pulmonary circulation improves after atrial septal defect (ASD) closure, the increasing left ventricular preload may contribute to mitral regurgitation (MR) deterioration. We aimed to evaluate the impact of MR after transcatheter ASD closure on clinical outcomes in adults. A total of 288 consecutive patients who underwent transcatheter ASD closure were enrolled. Changes in MR were assessed at 1 month after the procedure. The end point was defined as cardiovascular events. After the procedure, MR ameliorated in 3 patients and unchanged in 253, whereas MR deteriorated in 32. During a median follow-up of 24 months, patients with MR deterioration had no cardiovascular events, and the event-free survival rate was not different between patients with MR deterioration and those with MR amelioration or no-change (p = 0.355). Even in patients with MR deterioration, the New York Heart Association functional class improved after the procedure, with no cases of worsening functional class. Multivariate logistic regression analysis showed that MR deterioration was independently related to advanced age and female gender. The degree of enlargement of mitral valve annulus diameter after the procedure was greater in patients with MR deterioration than in those with MR amelioration or no-change, and it was correlated with the degree of MR deterioration. In conclusion, MR deterioration occurs in a minority of adult patients after transcatheter ASD closure; however, it is not linked with adverse outcomes. MR deterioration may be provoked by geometric changes in mitral valve annulus, especially in women with advanced age.

DOI： 10.1016/j.amjcard.2015.04.042

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

OBJECTIVES: This study assessed long-term outcome after transcatheter atrial septal defect (ASD) closure in older patients, especially those older than 75 years of age. BACKGROUND: The clinical benefits of transcatheter ASD closure in this aged population are controversial. METHODS: A total of 244 patients older than 50 years of age were divided into 3 groups according to age at procedure (50 to 59 years: n=69; 60 to 74 years: n=120; 75 years and older: n=55). The primary endpoint was defined as all-cause mortality and hospitalization due to heart failure or stroke. Improvements in functional capacity and cardiac remodeling after the procedure were also assessed. RESULTS: During a median follow-up of 36 months, mortality and hospitalization due to heart failure or stroke occurred in 18 patients (7%). Among patients older than 75 years of age, 2 died of noncardiovascular disease, 2 were hospitalized due to heart failure, and 1 had a stroke. More than 90% of patients older than 75 years of age did not experience these events. Kaplan-Meier analysis showed that the event-free survival rate was not different among the 3 age groups (log-rank test, p=0.780). New York Heart Association functional class and right ventricular/left ventricular end-diastolic diameter ratio improved in patients older than 75 years of age, similar to the other age groups. CONCLUSIONS: Long-term outcome after transcatheter ASD closure in patients older than 75 years of age is similar to that in the other, relatively younger age groups. This suggests that transcatheter ASD closure can be considered a valuable therapeutic option in patients older than 75 years of age.

DOI： 10.1016/j.jcin.2015.02.002

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

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BACKGROUND: Brugada syndrome (BrS)-type electrocardiogram (ECG) is concealed by complete right bundle-branch block (CRBBB) in some cases of BrS. Clinical significance of BrS masked by CRBBB is not well known. METHODS AND RESULTS: We reviewed an ECG database of 326 BrS patients who had type 1 ECG with or without pilsicainide. "BrS masked by CRBBB" was defined on ECG as <2-mm elevation of the J point at the time of CRBBB in the right precordial leads, and BrS-type J-point elevation ≥2 mm at the time of normalized QRS complex on relieved CRBBB. We identified 25 BrS patients (7.7%) with persistent (n=12) or intermittent CRBBB (n=13). Relief of CRBBB by pacing was performed in patients with persistent CRBBB. The prevalence of BrS masked by CRBBB was 3.1% (10/326 patients). Three patients had type 1 ECG, and 7 patients had type 2 or 3 ECG on relief of CRBBB. Two of these 10 patients had lethal arrhythmic events during the follow-up period (mean, 86.4±57.2 months). There was no prognostic difference between BrS masked by CRBBB and other BrS. CONCLUSIONS: In a small BrS population, CRBBB can completely mask typical BrS-type ECG. BrS masked by CRBBB is associated with the same risk of fatal ventricular tachyarrhythmia as other BrS.

DOI： 10.1253/circj.CJ-15-0618

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Language：Japanese   Publisher：Japan Heart Foundation  

DOI： 10.11281/shinzo.47.S1_33

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

Transcatheter closure of complex multiple atrial septal defects (ASDs) remains a challenge. We describe our clinical experience with staged device deployment in a patient with multiple ASDs using four Amplatzer septal occluder devices. Three-dimensional transesophageal echocardiography imaging contributed not only to the therapeutic guidance of successful device deployment but also to the decision making for the staged device delivery approach in a case of morphologically complex multiple ASDs.

DOI： 10.1111/echo.12731

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

Platypnea-orthodeoxia is a rare condition characterized by dyspnea and oxygen desaturation induced by the upright position and relieved by recumbency. The most common cause of this syndrome is right-to-left shunt through interatrial communications such as patent foramen ovale (PFO) or atrial septal defect (ASD). In addition, this syndrome can be caused by other extracardiac components, including pulmonary emphysema, pericardial disease, and prominent Eustachian valve. We experienced 3 cases of this syndrome, including 1 patient with PFO and 2 patients with ASD. Computer tomography imaging revealed aortic elongation and compression of the right atrium by ascending aorta in all of 3 patients. Transcatheter closure of PFO or ASD was successfully performed in all patients, including immediate improvements of symptoms and oxygen saturation without any complications.

DOI： 10.1007/s12928-014-0244-x

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Language：Japanese   Publisher：臨床心臓電気生理研究会  

症例は52歳、男性。以前より心電図にて早期再分極が認められていた。心室細動自然発作を来たしたため、当院入院。心電図ではI、II、aVl、aVf、V4-6にJ波を認めた。心臓電気生理学的検査において、多極カテーテルにて経冠静脈的に左室側壁心外膜側の電位を記録すると、単極および双極誘導において体表面心電図のJ波に相当する成分が記録され、この成分は心房頻回刺激およびisoproterenolにて減高し、pilsicainideにて増強を示した。一方、前室間静脈、中心静脈、左室側壁心内膜側からの記録ではJ波に相当する成分は記録されず、また心室プログラム刺激にて心室細動は右室心尖部、右室流出路、左室心内膜側からは誘発されず、左室側壁心外膜側からのみ誘発された。Pilsicainide投与後は左室心外膜側起源と思われる期外収縮が散発し、Brugada型波形は出現しなかった。本例はJ波症候群Type 1+2と考えられ、左室側壁心外膜側の電気的異常が病態の成因に関与することが示唆された興味深い1例と考えられたので報告する。(著者抄録)

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

Pericardial tamponade occurred 3 days after the catheter closure of an atrial septal defect (ASD) using Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN, USA). Before the closure, two-dimensional and real-time three-dimensional transesophageal echocardiography demonstrated a deficient aortic rim and atrial septal malalignment. Perforation of the right atrium toward the non-coronary sinus of the aortic root was confirmed at the emergent surgery. Cardiac erosion is one of the most catastrophic complications in ASD patients undergoing catheter closure with Amplatzer Septal Occluder. Hence, several risk factors for this complication are discussed and identified. Oversized device deployment and a deficient aortic rim are accepted factors potentially causing cardiac erosion. Besides, atrial septal malalignment, which is a morphological characteristic of ASD, may be a novel risk factor for cardiac erosion. <Learning objective: Cardiac erosion is a potentially lethal complication when catheter closure of atrial septal defects using Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN, USA) is provided to the patients. However, mechanisms of this complication remain to be completely elucidated. Atrial septal malalignment may be one of the novel risk factors for this catastrophic complication.>.

DOI： 10.1016/j.jccase.2013.12.004

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

DOI： 10.1016/j.hrthm.2013.10.057

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

Although numerous studies have shown an association between a patent foramen ovale (PFO) and cryptogenic cerebrovascular accidents (CVA), there has been no definitive control study that demonstrated the benefit of percutaneous device closure of a PFO compared to medical therapy in patients with CVA. Additionally, few clinical data exist for Japanese patients in this field. We demonstrate the initial experiences in catheter closure of a PFO as secondary prevention of CVA in Japan. Catheter closure of a PFO was attempted in 7 patients who were diagnosed with cryptogenic CVA. Mean age at the procedure was 54 ± 19 years. The presence of spontaneous interatrial right-to-left shunts was demonstrated by transesophageal contrast echocardiography without Valsalva maneuver in all of the patients. Amplatzer Cribriform device (n = 4) or Amplatzer PFO Occluder (n = 3) was used for the procedure and was successfully deployed. Device-related complications were not observed at the time of the procedure or during the follow-up period (mean period of 16 ± 9 months). Catheter closure of a PFO could be safely performed with Amplatzer Cribriform or Amplatzer PFO Occluder. This procedure may contribute to prevention of recurrent cryptogenic CVA in Japanese patients.

DOI： 10.1007/s12928-013-0193-9

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Language：Japanese   Publisher：Japan Heart Foundation  

DOI： 10.11281/shinzo.46.S3_203

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

症例は62歳、男性。早朝の苦悶様呼吸に続く呼吸停止疑いのため近医受診し、Brugada症候群疑いにて当院紹介入院。心電図では完全右脚ブロックを合併することもありBrugada型心電図との診断が容易ではなかった。電気生理学的検査時、ピルジカイニド1mg/kgを投与したが、やはり明瞭なBrugada型波形が得られないため、完全右脚ブロックの状態を是正する目的で右室心尖部より単発早期刺激を加えた。すると刺激間隔の短縮に伴いcoved型そしてsaddleback型の比較的明瞭なBrugada型心電図を得ることができた。完全右脚ブロックを伴いBrugada型心電図の診断が困難であったが右室心尖部単発早期断激によりBrugada型心電図が明瞭化され、また、相対的な右室伝導遅延の改善に伴いcoved型からsaddleback型に心電図が変化したBrugada症候群の1例を経験したので報告する。(著者抄録)

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2013&ichushi_jid=J00679&link_issn=&doc_id=20131216220028&doc_link_id=10.11281%2Fshinzo.45.S3_143&url=https%3A%2F%2Fdoi.org%2F10.11281%2Fshinzo.45.S3_143&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

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

OBJECTIVES: To evaluate the efficacy and safety of transcatheter closure of atrial septal defects (ASD) in patients over 70 years of age. BACKGROUND: Transcatheter closure of ASD is an established procedure in children and young adults, but the benefits of this procedure in geriatric patients are still unclear. METHODS: Between 2005 and 2010, 430 patients with ASD underwent transcatheter closure in our hospital. Among those patients, 30 consecutive patients older than 70 years of age were prospectively evaluated. RESULTS: Mean age at procedure was 75.8 ± 3.8 years (range: 70-85 years). Mean Qp/Qs was 2.4 ± 0.7 and mean ASD diameter was 20.3 ± 6.4 mm. Nine patients (30%) had a history of hospitalization due to heart failure. ASD closure was successfully performed in 28 patients (93%) without significant complications. During the follow-up period (mean period of 19.1 ± 11.3 months), New York Heart Association (NYHA) functional class was significantly improved in 20 patients (74%). Significant improvements of plasma BNP level, resting heart rate, and systolic pulmonary artery pressure were also observed. Improvement of tricuspid regurgitation was observed in 11 of 17 patients with moderate or severe regurgitation during the follow-up period. Conversely, worsening of mitral regurgitation was observed in 10 of the 27 patients. CONCLUSION: Transcatheter closure of ASD in geriatric patients can be performed safely. This procedure contributes to significant improvement of symptoms and positive cardiac remodeling. Long-term follow-up is mandatory, especially for patients with mitral regurgitation.

DOI： 10.1002/ccd.23457

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

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

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

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

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Language：Japanese   Publisher：(株)医学書院  

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

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Language：Japanese   Publisher：(株)ライフ・サイエンス  

心不全を合併した心房細動症例に対するアミオダロンの効果、安全性について検討した。心室性不整脈に対してアミオダロンを処方した124例を対象とした。処方時に発作性・持続性心房細動を合併していた症例は43例で、そのうち6ヵ月間経過観察できた心房細動症例は30例であった。アミオダロン投与による洞調律維持は18例で可能であった。洞調律維持群をsinus群、洞調律に回復しなかった群をAF群として検討した。LVEFはAF群では改善する傾向がみられた。心拍数はsinus群で低下傾向を示した。LADは両群ともに変化は認めなかった。BNP値はsinus群で有意に低下し、AF群では低下傾向を示した。KL-6は投与前後で有意差を認めず、KL-6が急上昇した1例は、CT所見で間質の線維化を認めたためアミオダロン投与を中止し、KL-6は投与前の数値にまで回復した。

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

A recent study has shown that cryptogenic stroke can occur even in patients with small or insignificant atrial septal defects (ASD). However, clinical experience in this field is still limited in Japan, also the efficacy and safety of catheter closure of such defects have not been identified. To evaluate the efficacy and safety of catheter closure of interatrial communication in patients with cryptogenic stroke, 13 patients who were diagnosed with cerebrovascular events due to cryptogenic embolism were included in this study. Mean age at procedure was 43 ± 15 (range 17-68) years. In all patients, the presence of spontaneous or provoked interatrial right-to-left shunts was demonstrated by transesophageal contrast echocardiography. Mean defect size evaluated by the balloon sizing technique was 9.2 ± 2.8 mm, and mean size of the Amplatzer Septal Occluder deployed was 9.5 ± 2.8 mm. Devices were successfully deployed in all patients, though one device migrated into the descending aorta was retrieved by a snare catheter. Complete closure was detected by transesophageal contrast echocardiography at 12 months after the procedure was in 11 (85%) of the 13 patients. During the follow-up period (30.1 ± 9.4 months), no recurrent thromboembolic event was observed. Catheter closure of interatrial right-to-left communications can be safely performed. This procedure may contribute to reduction or prevention of recurrent neurological events in this patient population.

DOI： 10.1007/s12928-011-0075-y

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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：臨床心臓電気生理研究会  

症例は39歳、男性。心室細動自然発作を認めるBrugada症候群との診断で当院入院。心電図ではV2でのsaddle back型ST上昇とI、II、aV1、V3-6でのJ波を認めた。心臓電気生理学的検査では、経冠静脈的に左室側壁心外膜側に多極カテーテルを挿入し電位の記録を行ったところ、単極誘導では明瞭なJ波が記録され、双極誘導ではJ波の成分に伴いout of QRS potentialが記録された。単極誘導でのJ波は心房ペーシング、およびisoproterenol投与で減高し、pilsicainide投与で増高した。また双極誘導でのout of QRS電位は心房ペーシングおよびisoproterenol投与で短縮しpilsicainide投与で延長した。再分極異常の性質を示す左室心外膜側J波がout of QRS potentialとしても記録され、J wave syndromeの成因を考えるうえで興味深い症例と考えられここに報告する。(著者抄録)

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Publishing type：Research paper (scientific journal)  

Objectives: The purpose of this study was to determine whether J wave is a marker of arrhythmic risk in patients with vasospastic angina (VSA). Background: Both VSA and the presence of J waves in ECG are associated with fatal ventricular arrhythmias and sudden death. However, the prevalence of J waves among VSA patients is little known. Methods: We reviewed 66 patients with VSA in our hospital retrospectively and assessed the association between the presence of J wave and clinical backgrounds among them. J wave was defined as an elevation of the QRS-ST junction of > 0.1mV from baseline. Results: Spontaneous VF and syncope occurred in 9 (13.6%) and 14 (21.2%) of all patients, respectively. J waves were observed in 33 patients (50%) and were significantly more prevalent in males than females (60.4% vs. 16.7%, Pμ0.01). Arrhythmic symptoms (ventricular arrhythmias or syncope) were observed frequently in patients with J waves, compared to without J wave (48.4% vs. 23.8%, Pμ0.05). Both of the locations and height of J waves were not associated with the history of arrhythmic symptoms. Conclusions: J waves are observed frequently among male patients with VSA. The presence of J waves potentially influence the development of lethal ventricular arrhythmias associated with VSA. © 2011, Japanese Heart Rhythm Society. All rights reserved.

DOI： 10.4020/jhrs.27.OP03_4

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

Transcatheter closure of atrial septal defects (ASDs) has become an accepted and reliable procedure. Although various complications have been recognized, tear of the atrial septal rim is a rare complication. We report a case of atrial septal rim tear that was diagnosed during the procedure by real-time three-dimensional transesophageal echocardiography (TEE). The device was successfully implanted 3 months after the initial intervention. RT3D TEE is more useful for displaying the entire shape of the defect and its spatial relationship (RT3D) with its neighboring structures compared with conventional two-dimensional echocardiography. By using both two-dimensional and RT3D TEE images, especially in cases with complicated ASD morphology, both the echocardiologist and interventionalist gain valuable information on the morphology of the ASD before and during the procedure.

DOI： 10.1016/j.echo.2010.04.004

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BACKGROUND: Mutations in SCN5A are reportedly linked to Brugada syndrome (BS), but recent observations suggest that they are not necessarily associated with ventricular fibrillation (VF) in BS patients. Therefore, the clinical importance of SCN5A mutations in BS patients was examined in the present study. METHODS AND RESULTS:  The 108 BS patients were examined for SCN5A mutations and various parameters were compared between patients with and without mutations. An implantable cardioverter defibrillator (ICD) was implanted in 49 patients and a predictor of appropriate ICD shock was investigated. The existence of a SCN5A mutation was not associated with initial VF episodes (21.7% vs 20.0%, P=0.373). In the secondary prevention group, appropriate shock-free survival rate was significantly lower in patients with spontaneous type 1 ECG than in those without (41.1% vs 85.7% at 2 years, P=0.014). The appropriate shock-free survival rate was also significantly lower in patients with SCN5A mutations than in those without (28.6% vs 83.3% at 1 year, P=0.040). Appropriate shock was more frequent in patients with SCN5A mutations than in those without (6.6±6.2 vs 1.7±3.0, P=0.007). CONCLUSIONS: SCN5A mutations are associated with early and frequent VF recurrence, but not with initial VF episodes. This is the first report on the genotype-phenotype interaction and clinical significance of this mutation.

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It has been reported that bepridil prevents ventricular fibrillation (VF) in patients with Brugada syndrome, but the comparative efficacy with and without mutation in the SCN5A gene has not been elucidated. The purpose of this study was to assess the efficacy of low-dose bepridil (100 mg/day) for VF prevention in patients with Brugada syndrome with and without SCN5A mutation. Among 130 patients with Brugada-type electrocardiogram (ECG), low-dose bepridil was administered to seven patients because of repetitive VF episodes, including three with and four without SCN5A mutation. Preventive effect for VF recurrence and changes of the ECG and the signal-averaged ECG were evaluated. Frequencies of VF episodes were reduced after treatment with low-dose bepridil in all three patients with the SCN5A mutation (before: 0.33 versus after: 0.02 episodes/month, P < 0.01), but not in all four patients without the SCN5A mutation (before: 0.43 versus after: 2.94 episodes/month, P = nonsignificant). Levels of ST-segment elevation at J points and duration of low-amplitude signals less than 40 µV in the terminal filtered QRS complex (LAS40) in signal-averaged ECG were improved exclusively in patients with the SCN5A mutation. Treatment with bepridil prevented recurrence of VF along with improvement of ST elevation and LAS40 in patients with Brugada syndrome with the SCN5A mutation.

DOI： 10.1097/FJC.0b013e3181f03c2f

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

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Language：Japanese   Publisher：臨床心臓電気生理研究会  

症例は32歳、女性。開胸術の既往があり、頻拍発作を主訴に来院。心電図にて心房頻拍を認めアブレーション目的に入院。CARTOを用いたvoltage mappingにて右房側壁は強く障害され、activation mappingにて右房内興奮は右房側壁を前方より後方へ伝播し、後壁を横断、中隔を後方から前方へ伝播した後、三尖弁-上大静脈間、いわゆるupper-loopと三尖弁-下大静脈間、いわゆるlower-loopを伝播した。以上より右房側壁にcommon channelを持ち、upper loopとlower loopを同時に旋回するdual-loop reentryと診断した。Common channelでfragmented electrogramが記録され、同部位の通電で速やかに頻拍は停止した。上大静脈と下大静脈周囲を興奮が各々旋回するまれなdual-loop reentryの1例と考える。(著者抄録)

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Language：Japanese   Publisher：(株)ライフ・サイエンス  

アミオダロン肺障害(AIPT)における臨床的特徴とアンギオテンシン系拮抗薬(ASA)の効果について検討した。対象は96例(男性74例、女性22例、平均年齢58.1歳)で、基礎疾患は拡張型心筋症39例、虚血性心疾患26例、肥大型心筋症10例、観察期間は平均33.8ヵ月であった。1)11例(11.5%)にAIPTが認められ、AIPT(+)群ではAIPT(-)群に比し繰り返す心不全のエピソードが有意に多かった。2)ヒト肺胞上皮細胞にアミオダロン添加でアポトーシスは濃度依存性に増加し、アミオダロンにアンギオテンシンII追加でとアポトーシスは有意に増強されたが、アンギオテンシンII受容体拮抗薬添加でアポトーシスは有意に抑制された。3)AIPT(+)群ではAIPT(-)群に比しASA投与量は有意に少なかった。多変量解析では繰り返す心不全エピソードとASA投与量が各々独立したAIPT発症の予測因子であった。

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

BACKGROUND: The aim of this study was to demonstrate the utility of real-time three-dimensional (3D) transesophageal echocardiography (RT3D-TEE) using a matrix array 3D transesophageal echocardiographic probe for morphologic evaluation and guidance of transcatheter closure of atrial septal defects (ASDs). METHODS: Forty-eight consecutive patients scheduled for the intervention were included. Two-dimensional (2D) transesophageal echocardiography (2D-TEE) and RT3D-TEE were performed before and during the procedures. Measurements of maximal ASD diameter and surrounding rims obtained on RT3D-TEE were compared with those obtained on 2D-TEE. RESULTS: In 46 patients (96%), optimal 3D images for the morphologic evaluation of ASDs were obtained. RT3D-TEE facilitated the evaluation of ASD morphology and surrounding rims and was able to provide intraprocedural information clearly. A Bland-Altman plot showed a mean maximal diameter difference of -0.12 mm between the means (95% limits of agreement, -2.2 to 2.5 mm). CONCLUSION: RT3D-TEE is a clinically useful, complementary option to 2D-TEE for evaluation of ASD morphology and for interventional guidance.

DOI： 10.1016/j.echo.2009.06.008

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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)日本不整脈心電学会  

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

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

OBJECTIVES: The aim of this study is to evaluate the feasibility and efficacy of device closure of atrial septal defect (ASD) in elderly patients with permanent atrial fibrillation. BACKGROUND: Little is known about the feasibility of device closure of ASD in those patients. METHODS: Nine consecutive patients (mean age 68.1 years) with permanent atrial fibrillation (>1 year persistent) underwent catheter closure using the Amplatzer septal occluder. Transthoracic echocardiography and plasma B-type natriuretic peptide (BNP) level were assessed before and at 24 hours; and 1, 3, and >6 months after the closure. Before the procedure, appropriate dose of warfarin was used in all, diuretics was used in 8/9. Same amount of medications were continued after the procedure. RESULTS: ASD could be closed in all (mean device size 27.3 mm) without hemodynamic and thromboembolic complications. New York Heart Association (NYHA) functional classification was significantly improved in all patients after device closure. No hemodynamic and thromboembolic complications were observed during the follow-up period (mean 10.6 months). Although permanent atrial fibrillation did not change in all after the procedure, resting heart rate decreased from 76.2 +/- 16.0 to 68.3 +/- 13.2 beats/min (P = 0.015). There was statistically significant improvement in right ventricular/left ventricular diameter ratio (1.08 +/- 0.16 to 0.73 +/- 0.10, P = 0.008) and plasma BNP level (183.7 +/- 90.5 to 94.6 +/- 47.4 pg/mL, P = 0.008) after >6 months device closure. CONCLUSIONS: Even in the patients complicated with permanent fibrillation, transcatheter closure of ASD can contribute to symptomatic improvement as well as cardiac geometric remodeling.

DOI： 10.1002/ccd.21870

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

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Language：Japanese   Publisher：(株)ライフ・サイエンス  

18ヵ月女児。発熱、嘔吐、下痢が出現した。哺乳がなくなり、ぐったりしているために近医を受診し、心電図にて頻脈性不整脈を指摘され、紹介受診した。発熱・腸炎などの感染症状や全周性の左室壁運動低下、急性心不全、さらに心筋逸脱酵素の上昇や頻脈性不整脈といった所見から、急性心筋炎と診断した。頻脈性不整脈のコントロールが困難で、血行動態は次第に不安定となったため、鎮静ならびに人工呼吸管理を行い、経皮的心肺補助装置を留置した。アミオダロンを開始し、頻拍は速やかに停止し、洞調律への復帰を認めた。急性心筋炎の治療としては、γグロブリン大量療法とステロイドパルス療法を施行した。徐脈傾向とQT延長の遷延に対して恒久的ペースメーカーを植え込み、軽快退院した。

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

【背景】ニフェカラント抵抗性の重症心室不整脈[心室頻拍・心室細動(VT/VF)]に対する静注アミオダロンの効果を自験例で検討した。【方法・結果】基礎心疾患は急性心筋梗塞6例、心筋炎2例、拡張型心筋症1例、肥大型心筋症1例で、心機能がきわめて低下していた症例であった。急性期に大動脈内バルーンポンプ(IABP)あるいは経皮的心肺補助装置(PCPS)などの補助循環が必要とされた症例は7例であった。使用したニフェカラントは、平均で初期投与0.39±0.15mg/kg、持続投与0.34±0.12mg/kgで、QTc時間は平均0.47→0.55secへ延長し、停止効果を7例(70%)に認めたが、頻拍再発抑制効果は認められなかったため、静注アミオダロンに切り替えた。8例では初期から少量持続投与(平均484±166mg/日)が行われた。アミオダロン単独でVT/VF停止効果を80%に、VT/VF再発抑制効果も80%に認められた。心拍数は100→71bpmとニフェカラント前値(95bpm)よりも減少していたが、ニフェカラントによって延長したQTcは0.55→0.47secと使用前値に戻っていた。4例が死亡したが不整脈死は認められなかった。アミオダロン投与にて血圧低下が起きた症例、房室ブロックが悪化した症例がそれぞれ1例認められた。多くの症例で薬物治療以外に、期外収縮を抑制する目的でペースメーカ、鎮静、呼吸管理などが行われていた。【結語】ニフェカラント抵抗性のVT/VF症例に対してアミオダロン静注は有効である可能性が高いが、徐脈・低血圧・房室ブロックといった副作用発現に注意が必要とされる。また、心不全に対する抗不整脈薬以外の治療もきわめて重要であると考えられた。(著者抄録)

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2009&ichushi_jid=J00681&link_issn=&doc_id=20090226210004&doc_link_id=%2Fdn4eleca%2F2009%2F002901%2F005%2F0026-0033%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdn4eleca%2F2009%2F002901%2F005%2F0026-0033%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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

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

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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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OBJECTIVES: The goal of our work was to examine the relationships of atrial fibrillation (AF) with genetic, clinical, and electrophysiological backgrounds in Brugada syndrome (BrS). BACKGROUND: Atrial fibrillation is often observed in patients with BrS and indicates that electrical abnormality might exist in the atrium as well as in the ventricle. SCN5A, a gene encoding the cardiac sodium channel, has been reported to be causally related to BrS. However, little is known about the relationships of atrial arrhythmias with genetic, clinical, and electrophysiological backgrounds of BrS. METHODS: Seventy-three BrS patients (49 +/- 12 years of age, men/women = 72/1) were studied. The existence of SCN5A mutation and clinical variables (syncopal episode, documented ventricular fibrillation [VF], and family history of sudden death) were compared with spontaneous AF episodes. Genetic and clinical variables were also compared with electrophysiologic (EP) parameters: atrial refractory period, interatrial conduction time (CT), repetitive atrial firing, and AF induction by atrial extra-stimulus testing. RESULTS: Spontaneous AF occurred in 10 (13.7%) of the BrS patients and SCN5A mutation was detected in 15 patients. Spontaneous AF was associated with higher incidence of syncopal episodes (60.0% vs. 22.2%, p < 0.03) and documented VF (40.0% vs. 14.3%, p < 0.05). SCN5A mutation was associated with prolonged CT (p < 0.03) and AF induction (p < 0.05) in EP study, but not related to the spontaneous AF episode and other clinical variables. In patients with documented VF, higher incidence of spontaneous AF (30.8% vs. 10.0%, p < 0.05), AF induction (53.8% vs. 20.0%, p < 0.03), and prolonged CT was observed. CONCLUSIONS: Spontaneous AF and VF are closely linked clinically and electrophysiologically in BrS patients. Patients with spontaneous AF have more severe clinical backgrounds in BrS. SCN5A mutation is associated with electrical abnormality but not disease severity.

DOI： 10.1016/j.jacc.2007.10.060

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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)日本不整脈心電学会  

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

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

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

症例は16歳男性。血圧低下をきたし前医搬送。冠動脈造影・左室造影・心筋生検を施行。好酸球性心筋炎と診断し3日間メチルプレドニゾロン(m-PSL)1g/日投与。その後PSLを減量するも好酸球数再増加を伴う血行動態悪化を認め、計3度のmPSL大量投与、γ-glb大量療法およびアザチオプリン投与を併用することで好酸球増多は認めなくなり心筋炎は改善した。その後PSL漸減可能となった。第27病日、感染契機に頻脈、血圧低下しPCPS挿入。第29病日PCPS挿入側の右下肢腫脹を認め減張切開施行。第45病日右下肢壊疽に対して右大腿切断術施行。第57病日小腸穿孔による急性汎発性腹膜炎をきたたし緊急手術を施行し小腸切除、ストーマ造設。その後感染コントロールが付いた後にPSLを漸減。PSL 5mgまで減量し、第188日ストーマ閉鎖。第211病日、リハビリ目的に前医転院となった。その後復学し現在外来通院中。ステロイド単剤では治療に難渋したが、アザチオプリンを追加投与することで治療し得た。(著者抄録)

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

DOI： 10.1016/S0735-1097(13)61914-2

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

DOI： 10.1016/j.amjcard.2012.01.227

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

DOI： 10.1016/S0735-1097(12)60242-3

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Language：English   Publishing type：Book review, literature introduction, etc.   Publisher：MDPI  

Background: Both nifekalant hydrochloride (NIF), a selective I-Kr blocker, and intravenous amiodarone (AMD), a multi-channel (including I-Kr blocking) blocker, have been reported to be efficacious for refractory arrhythmias. However, the optimal use of those antiarrhythmic drugs for refractory arrhythmia with severe heart failure has not been established. Intravenous AMD might be effective for arrhythmias refractory to NIF in patients with severe heart failure. Here, we report that intravenous amiodarone was effective in the treatment of nifekalant-resistant in a group of arrhythmia patients with severe heart failure. Methods: Eleven severe heart failure patients who had received intravenous AMD for treatment of NIF-resistant arrhythmias were included in this study, and retrospective analysis was performed. Clinical efficacy (terminative and preventive effects on arrhythmia) of intravenous AMD was evaluated. Results: All cases were emergent cases and had depressed left ventricular ejection fraction (30 +/- 13%). Clinical arrhythmias were ventricular fibrillation (VF) in four patients, ventricular tachycardia (VT) in six patients, and atrial fibrillation (AF) in one patient. NIF was administered to all patients by intravenous injection. After administration of NIF, VT/VF/AF was terminated in seven of the 10 patients, but a preventive effect was not obtained in any of the patients (NIF-resistance). Intravenous AMD (maintenance dose: 484 +/- 166 mg/day) was effective both in termination (80%) and in prevention (80%) of VT/VF events in those patients. It was also effective in termination (80%) and prevention (60%) of AF events refractory to NIF. During continuous AMD administration, no significant adverse effects or proarrhythmic effects were observed in any of the patients. Five patients died within one month, but there was no arrhythmic deaths. Conclusions: Intravenous AMD was effective in NIF-resistant lethal arrhythmias and was relatively safe in emergent cases with severe heart failure.

DOI： 10.3390/ph4060794

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

DOI： 10.1016/S0735-1097(11)61993-1

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

DOI： 10.1016/S0735-1097(11)61683-5

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A case is 77-year-old male. He was implanted dual chamber pacemaker due to complete atrioventricular block from left side in 1999. In 2006, he was referred to our hospital due to sustained ventricular tachycardia and left ventricular dysfunction. After several examinations, he was diagnosed as cardiac sarcoidosis and implanted cardiac resynchronization therapy with defibrillator (CRTD) from right side, because new lead could not pass between superior vena cava and innominate vein. In 2008, the scar on right side became reddish and swelling. We opened the scar, but we could not detect active infection. Then, we implanted new generator under right pectoral muscle. However, in 2011, he was diagnosed as pocket infection on right side without bacteremia and we extracted all 5 leads by Excimer laser. The pathogenic bacteria was Staphylococcus epidermidis. Because the patient was completely dependent on CRTD, single right ventricular pacing could not maintain hemodynamic. Then we employed dual chamber temporary pacing, which could maintain hemodynamic. Two weeks later, he was implanted new CRTD under left pectoral muscle. During follow up, any sign of device infection has not been appeared so far. © 2011, Japanese Heart Rhythm Society. All rights reserved.

DOI： 10.4020/jhrs.27.PJ1_105

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DOI： 10.1016/j.carpath.2010.02.002

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

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

特発性心室細動(IVF)には、Brugada症候群の心電図特徴を示さない例がある。原因や病態は複数と思われ、潜在性の心筋炎の可能性も考えられている。今回、当院で経験されたBrugada症候群が否定的なIVF12例について、その特徴を検討した。年齢は38±12歳、男性が8名(67%)。右室流出路起源の心室性期外収縮(PVC)から心室細動(VF)を起こした者が3名。連結期の短いPVCを認める者が3名。いずれも認めない者が6名であった。12誘導心電図で有意な所見は認めず、遅延電位は陰性。冠動脈に有意狭窄はなく、左室造影も正常。心臓電気生理学的検査(EPS)において、2例のみ右室の一部に軽度の異常電位を認めたが、その他の症例では認めていない。プログラム刺激による心室頻拍/心室細動(VT/VF)の誘発では、全例で持続するVT/VFは誘発されなかった。上記のことからIVFには器質的な異常は認めず、機能的な異常が原因となっている可能性が高いと考えられる。(著者抄録)

DOI： 10.11281/shinzo.41.S3_54

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

症例は31歳、女性。動悸発作を伴うWPW症候群にて他院で2回アブレーションを施行するも不成功であった。心臓電気生理検査では後中隔副伝導路を介する順方向性房室回帰性頻拍が容易に誘発された。洞調律中の検討では、副伝導路の心室付着部位は中心静脈(MCV)の遠位部で冠静脈(CS)との分岐部より約25mm離れていた。また、MCV内では洞調律中および右室ペーシング中に心房波と心室波の間にケント電位が記録された。しかしMCV内ではインピーダンス高値のため通電は断念した。次に右室ペーシング中の最早期心房興奮部位を検討したところ、CS入口部底部からその右房側に比較的広範に認められた。心房端が広く存在することが予想され、同部の電気的隔離を目的としてまずCS入口部側を焼灼し、次に三尖弁-下大静脈峡部を線状焼灼すると、副伝導路の両方向性伝導途絶が初めて得られた。心房付着部の電気的隔離により根治に成功した心外膜側後中隔副伝導路の1例を報告する。(著者抄録)

DOI： 10.11281/shinzo.41.S4_18

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2009&ichushi_jid=J00679&link_issn=&doc_id=20091221090003&doc_link_id=10.11281%2Fshinzo.41.S4_18&url=https%3A%2F%2Fdoi.org%2F10.11281%2Fshinzo.41.S4_18&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：Japanese   Publisher：(株)医学書院  

＜ポイント＞●心室頻拍の治療は,基礎心疾患の有無,頻拍中の血行動態によって異なる.●薬理作用と頻拍の機序を理解したうえでの,薬剤使用が不可欠である.●近年使用可能となったアミオダロン静注薬は,ニフェカラントと並び,心室性不整脈治療に非常に重要である.薬理作用の相違点を理解しておく必要がある.(著者抄録)

DOI： 10.11477/mf.1402103749

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

Web of Science

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

J-GLOBAL

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

CiNii Article

CiNii Books

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

CiNii Article

CiNii Books

J-GLOBAL

researchmap

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

J-GLOBAL

researchmap

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

researchmap

Language：Japanese   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Language：Japanese   Presentation type：Oral presentation (invited, special)  

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Presentation type：Symposium, workshop panel (nominated)  

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Language：English   Presentation type：Symposium, workshop panel (public)  

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

researchmap

Presentation type：Public lecture, seminar, tutorial, course, or other speech  

researchmap

Presentation type：Public lecture, seminar, tutorial, course, or other speech  

researchmap

Presentation type：Symposium, workshop panel (nominated)  

researchmap

Presentation type：Public lecture, seminar, tutorial, course, or other speech  

researchmap

researchmap

Presentation type：Public lecture, seminar, tutorial, course, or other speech  

researchmap