BACKGROUND:A growing number of patients with tetralogy of Fallot develop left ventricular systolic dysfunction and heart failure, in addition to right ventricular dysfunction. Although cardiac resynchronization therapy (CRT) is an established treatment option, the effect of CRT in this population is still not well defined. This study aimed to investigate the early and late efficacy, survival, and safety of CRT in patients with tetralogy of Fallot.METHODS:Data were analyzed from an observational, retrospective, multicenter cohort, initiated jointly by the Pediatric and Congenital Electrophysiology Society and the International Society of Adult Congenital Heart Disease. Twelve centers contributed baseline and longitudinal data, including vital status, left ventricular ejection fraction (LVEF), QRS duration, and NYHA functional class. Outcomes were analyzed at early (3 months), intermediate (1 year), and late follow-up (≥2 years) after CRT implantation.RESULTS:A total of 44 patients (40.3±19.2 years) with tetralogy of Fallot and CRT were enrolled. Twenty-nine (65.9%) patients had right ventricular pacing before CRT upgrade. The left ventricular ejection fraction improved from 32% [24%–44%] at baseline to 42% [32%–50%] at early follow-up (P<0.001) and remained improved from baseline thereafter (P≤0.002). The QRS duration decreased from 180 [160–205] ms at baseline to 152 [133–182] ms at early follow-up (P<0.001) and remained decreased at intermediate and late follow-up (P≤0.001). Patients with upgraded CRT had consistent improvement in left ventricular ejection fraction and QRS duration at each time point (P≤0.004). Patients had a significantly improved New York Heart Association functional class after CRT implantation at each time point compared with baseline (P≤0.002). The transplant-free survival rates at 3, 5, and 8 years after CRT implantation were 85%, 79%, and 73%.CONCLUSIONS:In patients with tetralogy of Fallot treated with CRT consistent improvement in QRS duration, left ventricular ejection fraction, New York Heart Association functional class, and reasonable long-term survival were observed. The findings from this multicenter study support the consideration of CRT in this unique population.

中文翻译：

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法洛四联症修复患者心脏再同步治疗的长期结果：一项多中心研究

背景：越来越多的法洛四联症患者除了右心室功能障碍外，还出现左心室收缩功能障碍和心力衰竭。尽管心脏再同步治疗 (CRT) 是一种既定的治疗选择，但 CRT 在此人群中的效果仍不明确。本研究旨在调查 CRT 对法洛四联症患者的早期和晚期疗效、生存率和安全性。 方法：对由儿科和先天性电生理学会和国际组织联合发起的观察性、回顾性、多中心队列的数据进行分析。成人先天性心脏病学会。十二个中心提供了基线和纵向数据，包括生命状态、左心室射血分数 (LVEF)、QRS 持续时间和 NYHA 功能分级。对CRT植入后早期（3个月）、中期（1年）和晚期（≥2年）随访结果进行分析。 结果：共有44例法洛四联症患者（40.3±19.2岁）接受CRT治疗。已注册。 29 名 (65.9%) 患者在 CRT 升级前进行了右心室起搏。左心室射血分数从基线时的 32% [24%–44%] 改善到早期随访时的 42% [32%–50%] ( P <0.001)，此后仍较基线有所改善 ( P ≤ 0.002)。 QRS 时限从基线时的 180 [160–205] ms 减少到早期随访时的 152 [133–182] ms（P <0.001），并在中后期随访时保持下降（P ≤0.001）。升级CRT的患者在每个时间点的左心室射血分数和QRS持续时间都有一致的改善（P≤0.004）。与基线相比，CRT 植入后每个时间点患者的纽约心脏协会功能分级均显着改善（P ≤0.002）。 CRT 植入后 3 年、5 年和 8 年的无移植生存率分别为 85%、79% 和 73%。 结论：在接受 CRT 治疗的法洛四联症患者中，QRS 时限、左心室射血分数、观察了纽约心脏协会的功能分级和合理的长期生存率。这项多中心研究的结果支持在这一独特人群中考虑 CRT。