記述言語：日本語   出版者・発行元：(公財)日本心臓財団  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Remote monitoring (RM) has been shown to reduce all-cause mortality in patients with implantable cardioverter-defibrillators or cardiac resynchronization therapy defibrillators (ICD/CRT-D). Not all devices transmit an alert for antitachycardia pacing (ATP) therapy, and it is unknown whether differences of RM alert affect the outcomes of electrical storm (ES). METHODS: We enrolled 42 patients with ICD/CRT-D whose out-of-hospital ES were detected by RM between 2013 and 2020. We divided their 54 episodes into two groups (ATP-alert-on; 22, ATP-alert-off; 32), and clinical outcomes were compared between the two groups. RESULTS: In 35 of 54 episodes of ES, ventricular tachycardia (VT) could be terminated within 24 h of ES onset just by ATP (ATP-alert-on: 14, ATP-alert-off: 21); however, many patients subsequently received shock delivery for VT. Among the 35 episodes, only in ATP-alert-on group, seven patients were prompted to visit our hospital without ICD shock through confirmation of ES by ATP-alert. Episodes that led to shock delivery 24 h or longer after the ES onset were significantly less common in the ATP-alert-on group (ATP-alert-on: 1/14, ATP-alert-off: 9/21, p = .03). Although there were no significant differences in the number of shock deliveries between episodes in the two groups, the number of ATP deliveries were significantly fewer in the ATP-alert-on group (12[7-26] vs. 29[16-53] in ATP-alert-off group, p = .03). Multivariate logistic regression analyses showed that the only ATP-alert significantly reduced ATP deliveries (HR = 0.14, 95%CI = 0.04-0.57, p = .003). CONCLUSION: Remote monitoring with an ATP-alert function during electrical storm may reduce appropriate ICD therapy through prompting early review.

DOI： 10.1111/pace.14334

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: The local conduction delay has been deemed to play an important role in the perpetuation of ventricular fibrillation (VF) in Brugada syndrome (BrS). We evaluated the relationship between the activation delay during programmed stimulation and cardiac events in BrS patients. METHODS: This study included 47 consecutive BrS patients who underwent an electrophysiological study and received implantable cardiac defibrillator therapy. We divided the patients into two groups based on whether they had developed VF (11 patients) or not (36 patients) during the follow-up period of 89 ± 53 months. The activation delay was assessed using the interval between the stimulus and the QRS onset during programmed stimulation. The mean increase in delay (MID) was used to characterize the conduction curves. RESULTS: The MID at the right ventricular outflow tract (RVOT) was significantly greater in patients with VF (4.5 ± 1.2 ms) than in those without VF (2.2 ± 0.9 ms) (p < .001). A receiver operating characteristics curve analysis indicated that the optimal cut-off point for discriminating VF occurrence was 3.3 with 88.9% sensitivity and 91.3% specificity. Furthermore, patients with an MID at the RVOT ≥ 3.3 ms showed significantly higher rates of VF recurrence than those with an MID at the RVOT < 3.3 ms (p < .001). The clinical characteristics, including the signal-averaged electrocardiogram measurement and VF inducibility were similar between the two groups. CONCLUSION: A prolonged MID at the RVOT was associated with VF and maybe an additional electrophysiological risk factor for VF in BrS patients.

DOI： 10.1111/jce.15065

PubMed

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記述言語：英語  

DOI： 10.1111/anec.12871

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: The significance of high precordial electrocardiograms in idiopathic ventricular fibrillation (IVF) is unknown. METHOD: This study included 50 consecutive patients (48 men; age, 42 ± 18 years) who had spontaneous ventricular fibrillation not linked to structural heart disease and received implantable cardiac defibrillator therapy. IVF was diagnosed in 35 patients and Brugada syndrome was diagnosed in other 15 patients. Electrocardiograms in high intercostal space were compared between 35 patients with IVF and 105 age- and sex-matched healthy controls (patient: control ratio, 1:3). RESULTS: The frequency of J point elevation ≥ 0.1 mV in the 4th intercostal spaces was similar between patients with IVF (14%) and healthy controls (7%). However, the frequency of J point elevation ≥ 0.1 mV in the 3rd intercostal space was higher in patients with IVF (40%) than controls (11%) (p < .01). J point elevation was present only in the 3rd intercostal space but not in the 4th intercostal space in 30% of patients with IVF but only in 6% of controls (p < .01). During follow-up, the recurrence of ventricular fibrillation was higher in patients with IVF who had J point elevation in the 3rd intercostal space (36%) and Brugada syndrome(40%) than those with IVF who did not have J point elevation in the 3rd intercostal space(11%) (p < .05 for both). CONCLUSION: J point elevation in the 3rd intercostal space was associated with IVF and recurrences of ventricular fibrillation. Electrocardiogram recordings in the high intercostal space may be useful to identify risk of sudden death.

DOI： 10.1111/anec.12820

PubMed

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Elsevier BV  

BACKGROUND: Real-world evidence of out-of-hospital ventricular fibrillation (VF), especially regarding intra-day change, remains unclear. We aimed to investigate that age- and gender-dependent difference of intra-day change of VF occurrence. METHOD: We enrolled 71,692 patients (males: 56,419 [78.7%], females: 15,273 [21.3%]) in whom cardiac VF had been documented from the 2005-2015 All-Japan Utstein Registry data. Subjects were divided into four groups: group-I (<18 years old), group-II (18-39), group-III (40-69), and group-IV (≥70). Among four groups in each of male and female, we compared the intra-day change of VF occurrence, and evaluated the risk factors of the unfavorable neurologic outcomes at 1 month after VF. RESULTS: Regardless of age, the incidence of VF was significantly greater in male than in female subjects. In male subjects, VF in group-I, III and IV occurred higher at daytime, however, group-II had no intra-day difference because group-II had a higher VF events at midnight~ early morning compared with other aged groups (Poisson regression analysis, p = .03). While in female, each group showed similar intra-day pattern of VF occurrence. Logistic regression analysis revealed that some of the clinical parameters such as time periods from call receipt to first shock and the presence of bystander cardiopulmonary resuscitation were important for risk of 30-day neurologically unfavorable outcomes. CONCLUSIONS: The intra-day change of VF occurrence was age-dependently different in males but not in females, suggesting age- and gender-dependent differences in underlying cardiac diseases. These might affect the significant difference in unfavorable neurologic outcome.

DOI： 10.1016/j.ijcard.2020.06.014

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Elsevier BV  

DOI： 10.1016/j.hrcr.2020.03.013

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Early repolarization syndrome is a recently proposed condition characterized by an early repolarization pattern in the electrocardiogram (ECG) and ventricular fibrillation in the absence of structural heart abnormalities. Although some studies have suggested that early repolarization is associated with frequency of atrial fibrillation, the association of early repolarization with atrial fibrillation is not well known. HYPOTHESIS: Early repolarization indicates the substrate for atrial fibrillation in addition to that for ventricular fibrillation. METHOD: This study included 79 patients (57 men [72%]; age, 45 ± 12 years) aged less than 60 years who had paroxysmal lone atrial fibrillation and 395 age- and sex-matched healthy controls (patient:control ratio, 1:5). Patients who had structural heart disease, hypertension, diabetes, hyperthyroidism, history of successful resuscitation, or the Brugada type ECG were excluded. ECGs recorded during sinus rhythm were compared between patients with atrial fibrillation and healthy controls. RESULTS: Early repolarization in the inferior and/or lateral leads was more common in patients with atrial fibrillation (25%) than controls (10%; P = 0.001). The location and magnitude of early repolarization were similar between the two groups. Other electrocardiographic measurements were not different between the two groups. Among patients with atrial fibrillation, there was no difference in clinical characteristics including age at atrial fibrillation development, sex, and body mass index between patients with early repolarization and those without early repolarization. Electrocardiographic measurements were not different between patients with early repolarization and those without early repolarization. CONCLUSION: Early repolarization was associated with lone atrial fibrillation. Early repolarization may indicate increased susceptibility to atrial fibrillation.

DOI： 10.1111/jce.13848

PubMed

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 60-year-old female with premature ventricular contractions (PVCs) originating from the bottom of the posteromedial papillary muscle of the left ventricle underwent radiofrequency catheter ablation (RFCA) using an irrigated-tip catheter. During ablation of the PVCs, a loud steam pop was observed. Intracardiac echocardiography (ICE) revealed a growing, hyperechogenic intramyocardial microbubble formation around the catheter tip. The formation disappeared slowly and completely, leaving an endocardial laceration without pericardial effusion. ICE imaging is valuable during a difficult RFCA procedure, because ICE reveals the exact anatomical position of the catheter and thus allows rapid evaluation of the occurrence of steam popping and any possible subsequent complication.

DOI： 10.1536/ihj.18-136

PubMed

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：日本語   出版者・発行元：(一社)日本内科学会  

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記述言語：日本語   出版者・発行元：(一社)日本内科学会  

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掲載種別：研究論文（学術雑誌）   出版者・発行元：Elsevier BV  

DOI： 10.1016/j.jacc.2017.10.076

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掲載種別：研究論文（学術雑誌）   出版者・発行元：Elsevier BV  

DOI： 10.1016/j.jelectrocard.2016.09.005

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease mainly caused by desmosome gene mutations. The genetic culprit, however, remains elusive in ∼50% of ARVC patients. One of the reasons for missing genetic abnormalities is the difficulty in detecting large deletions/duplications, which are called as copy number variation (CNV) by the Sanger sequencing method. This study aimed to identify CNVs in PKP2 and a part of other desmosome genes in ARVC patients. Methods and Results: The study cohort consisted of 71 ARVC probands who were diagnosed as definite or borderline cases based on 2010 Task Force Criteria. Among them, 32 (45%) carried at least one mutation in desmosome genes detected by the Sanger method. Using the multiplex ligation-dependent probe amplification method, we identified a male proband (1.4%) with a complete deletion of all PKP2 coding exons. He was 31 years old and showed exercise-induced sustained ventricular tachycardia with superior axis and left bundle-branch block pattern. His cardiac magnetic resonance imaging and computed tomography showed right ventricular dilatation and reduced ejection fraction. His 12-lead electrocardiogram showed T-wave inversion in V1-V3, and late potentials were positive, indicating definite ARVC. To confirm the precise location of the deletion, we performed relative quantitative PCR. We found complete deletion of both SYT10 and ALG10 located in 3' of PKP2; the total deletion size was at least 1.23 Mb. Conclusion: Screening for CNVs in desmosome genes is useful to identify the genetic basis of disease in clinically suspected ARVC patients.

DOI： 10.1093/europace/euw038

PubMed

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記述言語：日本語   出版者・発行元：公益財団法人 日本心臓財団  

<p> 75歳女性. 急性気管支炎のためクラリスロマイシン (CAM) 400mg/日の処方を受けたが, 失神をきたし救急搬送された. 心拍数40/分の徐脈性心房粗動と洞調律時の2 : 1房室ブロックに伴う高度のQT延長 (QTc=583～641ms) を認め, 複数回の失神を伴う多形性心室頻拍の発症を認めた. 電解質異常やたこつぼ型心筋症を示唆する壁運動異常, 虚血性心疾患は認めなかった. CAM休薬9日後には房室ブロックは改善し, QT間隔も正常化した (QT, QTc=425, 467ms). EPSにおいて, ニフェカラントを投与しQT延長を再現したところ, Wenckebach周期は500msから600msに延長し, 545ms周期ではAHブロックに伴う2 : 1房室ブロックが再現された. アトロピン投与で房室伝導の改善はなかった. 房室ブロック時の右室刺激では, Wenckebach周期で心室筋が捕捉された. クラリスロマイシンがQT延長とともに房室結節レベルでの伝導障害に関与し, 多形性心室頻拍発症を促進した可能性を示唆する症例を経験したため報告する.</p>

DOI： 10.11281/shinzo.48.S1_155

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記述言語：日本語   出版者・発行元：公益財団法人 日本心臓財団  

<p> 症例は49歳男性. 高血圧, 糖尿病のためカルシウム拮抗薬, インスリン治療を受けていた. 心電図では肥大性ST変化を認め, 心エコーでは壁厚15mmの対称性左室肥大を認められていたが, 左室収縮能は保持されていた. トラック運転中に意識消失し追突事故を起こした. 救急隊が250bpmの脈をふれない単形性心室頻拍 (VT) を確認し, 心肺蘇生中にVTは停止し回復した. 緊急搬送後の心臓精査にて冠動脈病変はなく, 心臓MRIで心室中隔中層の遅延造影所見を認めた.</p><p> 電気生理学的検査では血行動態が破綻するRBBB型とLBBB型の複数波形の単形性VTが誘発された. 一部のVTでは, エントレインメント所見を認めリエントリー機序が想定され, 心室中隔に拡張期電位を認めたことから同領域が不整脈基盤となっている可能性が示唆された.</p><p> 左室肥大例において心臓MRIで遅延造影所見を認める場合, 不整脈リスクを考慮すべきと考えられ, 文献的考察を交えて報告する.</p>

DOI： 10.11281/shinzo.47.S1_38

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記述言語：日本語   出版者・発行元：Japan Heart Foundation  

生来健康の27歳男性. 父親が57歳時に突然死している. 深夜飲酒中に心室細動をきたし当院に搬送された. 心肺蘇生, 低体温療法を施行し, 後遺症なく回復した. 心エコー, 心臓MRI, 冠動脈CTで器質的心疾患は認めなかった. Type 3 Brugada型心電図を認めたが, ICD植え込み後に施行したピルシカイニド負荷試験ではtype 1様波形変化が1誘導のみであり, 特発性心室細動と診断した. また, 同検査中に右脚ブロック, 左軸偏位型の心室性期外収縮を認めた. 運動負荷試験後の夜間から早朝にこの心室性期外収縮より心室細動が惹起され, ショック作動を繰り返した. 心臓電気生理検査では左室基部後壁心外膜側に心室性期外収縮の起源を認め, 心内膜側から焼灼を試みたが, 完全には抑制されなかった. その後, ベプリジル内服により心室性期外収縮, 心室細動は抑制され, 以後9カ月間再発なく経過している. 心室細動を惹起する心室性期外収縮の起源が同定され, bepridilが奏功した特発性心室細動症例を経験したので文献的考察を加えて報告する.

DOI： 10.11281/shinzo.46.S2_56

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：日本語   出版者・発行元：(公財)日本心臓財団  

27歳時に片側睾丸摘出の既往がある61歳男性。母親とその姉妹2人が20歳頃に突然死している。失神や多形性心室頻拍(TdP)の既往はなかったが、50歳頃より検診でQT延長を指摘されていた。今回、運転中に初めて失神し搬送された。この際、著明なQT延長(QTc=666ms)とTdP頻発を認めた。器質的心疾患は認めず、家族歴から先天性QT延長症候群と考えられたが、本例では60歳代での心原性失神の初発であった。検診心電図では、QT延長は段階的に顕性化していた。本例では数日前からエフェドリン含む感冒薬の内服歴がありTdP発症の要因となったと考えられた。さらに本例では明らかなテストステロン低値を認めており、QT延長症候群の高齢での顕性化に寄与したと考えられた。QT延長例や潜在的素因を持つ例に内分泌的異常や薬剤など幅広い修飾因子が加わることで、高齢で初回発作をきたす例があり注意を要する。(著者抄録)

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記述言語：日本語   出版者・発行元：(公財)日本心臓財団  

CRT-D植込み後、左室リード断線による心不全悪化をきたし、冠静脈狭窄のため異なる部位への再留置を余儀なくされた3症例を経験した。3例とも左室リードは単純牽引で用手的抜去可能であった。症例1:68歳女性。拡張型心筋症(DCM、EF=37%、QRS=132ms、NYHAIII)、徐脈性心房細動、心室頻拍のためCRT-Dを植込み、NYHAIIに改善した。植込み8年2月後に左室リード断線をきたした。後側壁側から側壁側への再留置となり、その後初回留置後より利尿剤の減量が可能となった。症例2:71歳男性。DCM(EF=32%、QRS=180ms、NYHAIII)のためCRT-Dを植込み、NYHAIIに改善した。2年3ヵ月後に左室リード断線をきたした。心室刺激閾値高値や横隔神経捕捉のため目標領域への留置が困難であり、中心静脈経由で後側壁側に再留置したが、心不全は改善せず術後54日目に死亡した。症例3:57歳女性。DCM(EF=17%、QRS=190ms、NYHAIII)のためCRT-Dを植込みNYHAIIに改善した。4年7ヵ月後に左室リード断線をきたした。前側壁側から前壁側への再留置となり、その後心不全が悪化した(NYHAIII)。結語:左室リードの再留置時には、同部位への留置が困難となる可能性、他部位への留置によりCRTの効果が異なる可能性を考慮する必要がある。(著者抄録)

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記述言語：日本語   出版者・発行元：新潟医学会  

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その他リンク： http://search.jamas.or.jp/link/ui/2014333889

記述言語：日本語   出版者・発行元：ライフサイエンス出版(株)  

70歳男性。7年前に急性側壁心筋梗塞に対しPCIを施行された際に心エコーでEF35%、僧房弁逆流(MR)は中等度、三尖弁逆流の圧較差(TRPG)は21.9mmHgであった。その後、心不全状態の増悪に加え心拡大の進行、MRの増悪とTRPGの上昇が認められため入院となった。胸部X線ではCTRが60%ほか、近位肺動脈の拡大が認められ、心エコーでは左室壁運動はびまん性に低下し側壁は無動であった。一方、肺血流シンチグラフィでは右肺中葉が区域性に集積が乏しく、心カテーテル検査では冠動脈造影で有意狭窄はなく、肺動脈造影では狭窄や途絶はみられなかった。また、経肺圧較差が12mmHgを超え、out of proportion PHの病態であった。以上、これらの検査所見から本症例は心筋梗塞後の機能的僧房弁閉鎖不全症に伴う肺高血圧症(PH)と考えられた。そこで、治療にあたっては重度の僧房弁閉鎖不全から手術適応であるものの重度の肺高血圧も呈していることから手術はハイリスクであり、シルデナフィルの内服を開始した。その結果、60mgまで増量により肺うっ血の増悪は認めず心不全は改善した。以後、僧房弁閉鎖不全に対する手術待機として一旦退院となったが、左心不全が増悪して再入院となった。右心カテーテル検査ではout of proportion PHの病態に戻っており、内科的治療での管理は不可能と判断され、僧房弁形成術+三尖弁縫縮術が施行された。目下、外来通院中であるがTRPGの増悪なく経過は良好である。

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