Clinic for Electrophysiology, Herz- und Diabeteszentrum NRW, Ruhr-Universität Bochum, Georgstr.11, 32545 Bad Oeynhausen, Germany.
Electrophysiology Center Bremen, Bremen, Germany.
Europace. 2019 Jun 1;21(6):961-969. doi: 10.1093/europace/euz016.
Non-compaction cardiomyopathy (NCCM) is associated with high rates of mortality and morbidity. Knowledge regarding risk stratification, arrhythmogenesis, therapy, and prognosis is limited. The aim of this study was to analyse the outcome of patients suffering from NCCM and ventricular arrhythmias (VAs) focusing on a treatment with implantable cardioverter-defibrillator (ICD) therapy and catheter ablation.
We conducted a multicentre observational study on 18 patients with NCCM, who underwent ICD implantation for secondary (n = 12) and primary (n = 6) prevention. In patients with multiple symptomatic episodes of VAs catheter ablation was performed. During a follow-up of 62 ± 42 months, 12 patients (67%) presented with appropriate ICD therapies [ventricular tachycardia (VT): n = 8; ventricular fibrillation (VF): n = 4; VT/VF: n = 3]. Ten patients underwent catheter ablation for VT/VF. Solely endocardial ablation was conducted in eight patients, and in two patients endo- and epicardial ablation was performed within the same procedure. Acute procedural success was achieved in 9/10 patients. Ventricular tachycardia recurrence was observed in two patients and the median arrhythmia free interval was 9.5 months (interquartile range 5.3-21 months). One patient underwent reablation, four patients died due to the underlying NCCM, and one patient received a left ventricular assist device.
Ventricular arrhythmias are common in patients suffering from NCCM and ICD therapy may be effective for primary and secondary prevention. In our cohort, consisting of patients with multiple VA episodes and recurrent ICD therapy, catheter ablation offered a safe and effective therapeutically option.
非致密性心肌病（NCCM）与高死亡率和发病率相关。关于风险分层、心律失常发生机制、治疗和预后的知识有限。本研究旨在分析患有 NCCM 和室性心律失常（VA）患者的结局，重点关注植入式心脏复律除颤器（ICD）治疗和导管消融治疗。
我们对 18 名接受 ICD 植入治疗的 NCCM 患者进行了一项多中心观察性研究，这些患者植入 ICD 的目的是二级（n=12）和一级（n=6）预防。对于有多次症状性 VA 发作的患者，进行了导管消融治疗。在 62±42 个月的随访期间，12 名患者（67%）出现了适当的 ICD 治疗[室性心动过速（VT）：n=8；心室颤动（VF）：n=4；VT/VF：n=3]。10 名患者因 VT/VF 接受了导管消融治疗。8 名患者仅进行了心内膜消融，2 名患者在同一手术中进行了心内和心外膜消融。9/10 名患者在急性手术中获得了成功。2 名患者出现 VT 复发，心律失常无复发间隔的中位数为 9.5 个月（四分位间距 5.3-21 个月）。1 名患者接受了再次消融，4 名患者因 NCCM 死亡，1 名患者接受了左心室辅助装置。
VA 在患有 NCCM 的患者中很常见，ICD 治疗可能对一级和二级预防有效。在我们的队列中，包括多次 VA 发作和反复 ICD 治疗的患者，导管消融提供了一种安全有效的治疗选择。
