1st Department of Medicine - Cardiology, University Medical Centre Mannheim, Germany; Partner Site Heidelberg/Mannheim, German Center for Cardiovascular Research (DZHK), Mannheim, Germany.
Hannover Heart Rhythm Center, Department of Cardiology and Angiology, Hannover Medical School, Carl-Neuberg-Str. 1, 30625 Hannover, Germany.
Int J Cardiol. 2021 Jan 15;323:100-105. doi: 10.1016/j.ijcard.2020.08.089. Epub 2020 Aug 29.
Inherited arrhythmia syndromes and genetic cardiomyopathies attribute in a significant proportion to sudden cardiac death. Implantable cardioverter defibrillators (ICDs) are the cornerstone in the prevention of sudden death in high-risk patients. However, ICD therapy is also associated with high rates of inappropriate shocks and/or device-related complications especially in young patients.
To determine the outcome of high-risk patients with inherited arrhythmia syndromes and genetic cardiomyopathies comparing two defibrillator technologies.
Between 2010 and 2018, 183 consecutive patients from two large German tertiary care centers were enrolled in the study. The majority of patients (83%) had either cardiac channelopathies or idiopathic ventricular fibrillation without cardiac structural abnormalities, while the remaining 17% had a genetic cardiomyopathy (HCM/ARVC). Eighty-six patients (47%) received a transvenous ICD (TV-ICD), while a subcutaneous ICD (S-ICD) was implanted in another 97 patients (53%).
During a mean follow-up of 4.3 years, 30 patients had an appropriate ICD therapy (annual rate 3.8%). Fifteen patients experienced an inappropriate shock (annual rate 1.9%). Lead failure occurred in 17 (9%) patients and was less frequent in the S-ICD group (OR 0.48, 95%CI 0.38-0.62). Adverse defibrillator events, defined as a composite of inappropriate shocks and lead failure requiring surgical revision were significantly lower in the S-ICD group as compared to the TV-ICD group (OR 0.55, 95%CI 0.41-0.72). There was a non-significant trend towards lower appropriate shocks in the S-ICD group, that in combination with all-cause shocks yielded in a significantly higher freedom of any shock in the S-ICD group (RR 39%, p = 0.003). No deaths occurred during follow-up.
The present data favor the use of the subcutaneous ICD for patients with inherited arrhythmia syndromes and genetic cardiomyopathies who do not need anti-bradycardia pacing.
遗传性心律失常综合征和遗传性心肌病在很大程度上导致了心源性猝死。植入式心脏复律除颤器（ICD）是预防高危患者心源性猝死的基石。然而，ICD 治疗也与高比例的不适当电击和/或器械相关并发症相关，尤其是在年轻患者中。
比较两种除颤器技术在遗传性心律失常综合征和遗传性心肌病高危患者中的疗效。
2010 年至 2018 年间，来自德国两家大型三级护理中心的 183 例连续患者入组本研究。大多数患者（83%）患有心脏通道病或特发性室颤，无心脏结构异常，而其余 17%的患者患有遗传性心肌病（HCM/ARVC）。86 例患者（47%）接受了经静脉 ICD（TV-ICD）治疗，而另外 97 例患者（53%）接受了皮下 ICD（S-ICD）治疗。
平均随访 4.3 年后，30 例患者接受了适当的 ICD 治疗（年发生率 3.8%）。15 例患者发生不适当电击（年发生率 1.9%）。17 例患者出现导联故障（9%），S-ICD 组发生率较低（OR 0.48，95%CI 0.38-0.62）。与 TV-ICD 组相比，S-ICD 组不良除颤事件（定义为不适当电击和导联故障的复合事件，需要手术修正）明显较低（OR 0.55，95%CI 0.41-0.72）。S-ICD 组适当电击的发生率呈下降趋势，但与全因电击结合后，S-ICD 组无任何电击的发生率显著更高（RR 39%，p=0.003）。随访期间无死亡发生。
目前的数据支持对不需要抗心动过缓起搏的遗传性心律失常综合征和遗传性心肌病患者使用皮下 ICD。
