致心律失常性右室心肌病伴持续性室性心动过速患者中左、右束支阻滞形态室性心动过速的发生率：来自欧洲致心律失常性心肌病调查的结果。

The prevalence of left and right bundle branch block morphology ventricular tachycardia amongst patients with arrhythmogenic cardiomyopathy and sustained ventricular tachycardia: insights from the European Survey on Arrhythmogenic Cardiomyopathy.

机构信息

Heart Institute, Hadassah University Hospital, Kalman Ya'Akov Man Street, 9112001, Jerusalem, Israel.

Sackler School of Medicine, Tel Aviv University, P.O. Box 39040, Tel Aviv 6997801, Israel.

出版信息

Europace. 2022 Feb 2;24(2):285-295. doi: 10.1093/europace/euab190.

DOI:10.1093/europace/euab190

PMID:34491328

Abstract

AIMS

In arrhythmogenic cardiomyopathy (ACM), sustained ventricular tachycardia (VT) typically displays a left bundle branch block (LBBB) morphology while a right bundle branch block (RBBB) morphology is rare. The present study assesses the VT morphology in ACM patients with sustained VT and their clinical and genetic characteristics.

METHODS AND RESULTS

Twenty-six centres from 11 European countries provided information on 954 ACM patients who had ≥1 episode of sustained VT spontaneously documented during patients' clinical course. Arrhythmogenic cardiomyopathy was defined according to the 2010 Task Force Criteria, and VT morphology according to the QRS pattern in V1. Overall, 882 (92.5%) patients displayed LBBB-VT alone and 72 (7.5%) RBBB-VT [alone in 42 (4.4%) or in combination with LBBB-VT in 30 (3.1%)]. Male sex prevalence was 79.3%, 88.1%, and 56.7% in the LBBB-VT, RBBB-VT, and LBBB + RBBB-VT groups, respectively (P = 0.007). First RBBB-VT occurred 5 years after the first LBBB-VT (46.5 ± 14.4 vs 41.1 ± 15.8 years, P = 0.011). An implanted cardioverter-defibrillator was more frequently implanted in the RBBB-VT (92.9%) and the LBBB + RBBB-VT groups (90%) than in the LBBB-VT group (68.1%) (P < 0.001). Mutations in PKP2 predominated in the LBBB-VT (65.2%) and the LBBB + RBBB-VT (41.7%) groups while DSP mutations predominated in the RBBB-VT group (45.5%). By multivariable analysis, female sex was associated with LBBB + RBBB-VT (P = 0.011) while DSP mutations were associated with RBBB-VT (P < 0.001). After a median follow-up of 103 (51-185) months, death occurred in 106 (11.1%) patients with no intergroup difference (P = 0.176).

CONCLUSION

RBBB-VT accounts for a significant proportion of sustained VTs in ACM. Sex and type of pathogenic mutations were associated with VT type, female sex with LBBB + RBBB-VT, and DSP mutation with RBBB-VT.

摘要

目的

在致心律失常性右室心肌病（ACM）中，持续性室性心动过速（VT）通常表现为左束支传导阻滞（LBBB）形态，而右束支传导阻滞（RBBB）形态较为少见。本研究评估了具有持续性 VT 的 ACM 患者的 VT 形态及其临床和遗传特征。

方法和结果

来自 11 个欧洲国家的 26 个中心提供了 954 名 ACM 患者的信息，这些患者在其临床病程中自发性记录到≥1 次持续性 VT。致心律失常性心肌病根据 2010 年工作组标准进行定义，VT 形态根据 V1 中的 QRS 模式进行判断。总体而言，882 名（92.5%）患者单独出现 LBBB-VT，72 名（7.5%）患者出现 RBBB-VT[单独出现 42 名（4.4%）或与 LBBB-VT 同时出现 30 名（3.1%）]。在 LBBB-VT、RBBB-VT 和 LBBB+RBBB-VT 组中，男性患病率分别为 79.3%、88.1%和 56.7%（P=0.007）。首次出现 RBBB-VT 比首次出现 LBBB-VT 晚 5 年（46.5±14.4 岁 vs 41.1±15.8 岁，P=0.011）。植入式心脏复律除颤器在 RBBB-VT（92.9%）和 LBBB+RBBB-VT（90%）组中比在 LBBB-VT 组（68.1%）更频繁地植入（P<0.001）。PKP2 突变在 LBBB-VT（65.2%）和 LBBB+RBBB-VT（41.7%）组中更为常见，而 DSP 突变在 RBBB-VT 组中更为常见（45.5%）。多变量分析显示，女性与 LBBB+RBBB-VT 相关（P=0.011），而 DSP 突变与 RBBB-VT 相关（P<0.001）。中位随访 103（51-185）个月后，106 名（11.1%）患者死亡，各组间无差异（P=0.176）。