Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom.
Division of Pediatric Cardiology, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital-IRCSS, Rome, Italy.
Heart. 2020 Jul;106(14):1052-1058. doi: 10.1136/heartjnl-2019-316202. Epub 2020 Apr 8.
The systemic right ventricle (SRV), defined as the morphological right ventricle supporting the systemic circulation, is relatively common in congenital heart disease (CHD). Our review aimed at examining the current evidence, knowledge gaps and technical considerations regarding implantable cardiac electronic device therapy in patients with SRV. The risk of sinus node dysfunction (SND) after atrial switch repair and/or complete heart block in congenitally corrected transposition of great arteries requiring permanent pacing increases with age. Similar to acquired heart disease, indication for pacing includes symptomatic bradycardia, SND and high degree atrioventricular nodal block. Right ventricular dysfunction and heart failure also represent important complications in SRV patients. Cardiac resynchronisation therapy (CRT) has been proposed to improve systolic function in SRV patients, although indications for CRT are not well defined and its potential benefit remains uncertain. Amongst adult CHD, patients with SRV are at the highest risk for sudden cardiac death (SCD). Nevertheless, risk stratification for SCD is scarce in this cohort and implantable cardioverter-defibrillator indication is currently limited to secondary prevention. Vascular access and the incidence of device-related complications, such as infections, inappropriate shocks and device system failure, represent additional challenges to implantable cardiac electronic device therapy in patients with SRV. A multidisciplinary approach with tertiary expertise and future collaborative research are all paramount to further the care for this challenging nonetheless ever increasing cohort of patients.
系统性右心室（SRV）是指支持体循环的形态右心室，在先天性心脏病（CHD）中较为常见。我们的综述旨在检查目前关于植入式心脏电子设备治疗 SRV 患者的证据、知识空白和技术考虑因素。房室结功能障碍（SND）的风险在接受心房调转修复和/或完全性大动脉转位的患者中随着年龄的增长而增加，需要永久性起搏。与获得性心脏病相似，起搏的指征包括有症状的心动过缓、SND 和高度房室结阻滞。右心室功能障碍和心力衰竭也是 SRV 患者的重要并发症。心脏再同步治疗（CRT）已被提出用于改善 SRV 患者的收缩功能，尽管 CRT 的指征尚未明确，其潜在益处仍不确定。在成人 CHD 中，SRV 患者发生心源性猝死（SCD）的风险最高。然而，在这一人群中，SCD 的风险分层很少，植入式心脏除颤器的指征目前仅限于二级预防。血管通路和器械相关并发症的发生率，如感染、不适当的电击和器械系统故障，是对 SRV 患者植入式心脏电子设备治疗的额外挑战。多学科方法、三级专业知识和未来的合作研究对于进一步治疗这一具有挑战性但日益增加的患者群体至关重要。
