Koepsel Katharina, Dreher Tobias C, Blockhaus Christian, Gotzmann Michael, Klein Norbert, Kuntz Thomas, Shin Dong-In, Lapp Hendrik, Schiedat Fabian, Abumayyaleh Mohammad, Beiert Thomas, Weth Christian, Kovacs Boldizsar, Rosenkaimer Stephanie, Kowitz Jacqueline, Saguner Ardan Muammer, Erath Julia W, Duru Firat, Mügge Andreas, Akin Ibrahim, Aweimer Assem, Hamdani Nazha, El-Battrawy Ibrahim
Department of Cellular and Translational Physiology and Institute für Forschung und Lehre (IFL), Institute of Physiology, Molecular and Experimental Cardiology, Ruhr-University Bochum, Bochum, Germany.
Department of Cardiology and Angiology, Bergmannsheil University Hospital, Ruhr University of Bochum, Bochum, Germany.
Front Cardiovasc Med. 2024 Jul 10;11:1384736. doi: 10.3389/fcvm.2024.1384736. eCollection 2024.
Data on the use of the wearable cardioverter defibrillator in patients suffering from inherited and congenital heart disease are limited. Consequently, evidence for guideline recommendations in this patient population is lacking.
In total 1,675 patients were included in a multicenter registry of eight European centers. In the present cohort, we included 18 patients suffering from congenital and inherited heart disease.
Nine patients (50%) were male with a mean age of 41.3 ± 16.4 years. Four patients suffered from hypertrophic cardiomyopathy (HCM), four patients suffered from non-compaction cardiomyopathy (NCCM), two patients were diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC) and one patient suffered from muscular dystrophy of the limb-girdle type with cardiac involvement, secondary cardiomyopathy. Three patients presented with Brugada syndrome (BrS). One patient suffered from long-QT syndrome type 1 (LQTS1). Furthermore, two patients had congenital heart defects and one patient suffered from cardiac sarcoidosis (CS). There were no appropriate/inappropriate shocks with the WCD in this cohort. One patient had recurrent self-limiting sustained ventricular tachycardia during the wear time, but actively inhibited a shock and was hospitalized. The compliance rate in this cohort was 77.8% with a mean wear time of 45.3 ± 26.9 days with a mean follow-up time of 570 ± 734 days. 55.6% (10/18) of the patients received an ICD after WCD wear time.
This retrospective study of patients with inherited and congenital heart disease shows that WCD use is not beneficial in the majority of patients with inherited and congenital heart disease.
关于可穿戴式心脏复律除颤器在患有遗传性和先天性心脏病患者中的应用数据有限。因此，这一患者群体中缺乏指南推荐的证据。
共有1675例患者纳入了欧洲八个中心的多中心注册研究。在本队列中，我们纳入了18例患有先天性和遗传性心脏病的患者。
9例（50%）为男性，平均年龄41.3±16.4岁。4例患有肥厚型心肌病（HCM），4例患有心肌致密化不全心肌病（NCCM），2例被诊断为致心律失常性右室心肌病（ARVC），1例患有伴有心脏受累的肢带型肌营养不良症、继发性心肌病。3例出现布加综合征（BrS）。1例患有1型长QT综合征（LQTS1）。此外，2例有先天性心脏缺陷，1例患有心脏结节病（CS）。本队列中使用可穿戴式心脏复律除颤器（WCD）期间未发生恰当/不恰当电击。1例患者在佩戴期间出现复发性自限性持续性室性心动过速，但主动抑制了电击并住院治疗。本队列的依从率为77.8%，平均佩戴时间为45.3±26.9天，平均随访时间为570±734天。55.6%（10/18）的患者在WCD佩戴期后接受了植入式心脏复律除颤器（ICD）。
这项对遗传性和先天性心脏病患者的回顾性研究表明，对于大多数遗传性和先天性心脏病患者而言，使用WCD并无益处。
