Université de Paris, Institut National de la Santé et de la Recherche Médicale, Paris Cardiovascular Research Centre, France (V.W., A.A., S.B., M. Ladouceur, K.N., M.-C.P., A.S., X.J., E.M.).
Adult Congenital Heart Disease Medico-Surgical Unit (V.W., L.I., M. Ladouceur), European Georges Pompidou Hospital, Paris, France.
Circulation. 2020 Oct 27;142(17):1612-1622. doi: 10.1161/CIRCULATIONAHA.120.046745. Epub 2020 Oct 1.
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce.
A Nationwide French Registry including all patients with tetralogy of Fallot with an ICD was initiated in 2010 by the French Institute of Health and Medical Research. The primary time to event end point was the time from ICD implantation to first appropriate ICD therapy. Secondary outcomes included ICD-related complications, heart transplantation, and death. Clinical events were centrally adjudicated by a blinded committee.
A total of 165 patients (mean age, 42.2±13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (interquartile range) follow-up of 6.8 (2.5-11.4) years, 78 (47.3%) patients received at least 1 appropriate ICD therapy. The annual incidence of the primary outcome was 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively; =0.03). Overall, 71 (43.0%) patients presented with at least 1 ICD complication, including inappropriate shocks in 42 (25.5%) patients and lead dysfunction in 36 (21.8%) patients. Among 61 (37.0%) patients in primary prevention, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with, respectively, 0, 1, 2, or ≥3 guidelines-recommended risk factors. QRS fragmentation was the only independent predictor of appropriate ICD therapies (hazard ratio, 3.47 [95% CI, 1.19-10.11]), and its integration in a model with current criteria increased the 5-year time-dependent area under the curve from 0.68 to 0.81 (=0.006). Patients with congestive heart failure or reduced left ventricular ejection fraction had a higher risk of nonarrhythmic death or heart transplantation (hazard ratio, 11.01 [95% CI, 2.96-40.95]).
Patients with tetralogy of Fallot and an ICD experience high rates of appropriate therapies, including those implanted in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria including QRS fragmentation might improve risk stratification. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03837574.
法洛四联症（TOF）是最常见的发绀型先天性心脏病，心脏性猝死是这些患者的重要死亡模式。评估植入式心脏复律除颤器（ICD）在这一患者群体中的数据仍然很少。
2010 年，法国健康与医学研究所发起了一项全国性的法国法洛四联症合并 ICD 患者注册研究。主要的时间事件终点是从 ICD 植入到首次适当 ICD 治疗的时间。次要结局包括 ICD 相关并发症、心脏移植和死亡。临床事件由一个盲法委员会进行中心裁决。
共纳入来自 40 个中心的 165 例患者（平均年龄 42.2±13.3 岁，70.1%为男性），其中 104 例（63.0%）为二级预防。中位（四分位间距）随访 6.8（2.5-11.4）年，78 例（47.3%）患者至少接受了 1 次适当的 ICD 治疗。主要结局的年发生率为 10.5%（一级预防和二级预防分别为 7.1%和 12.5%；=0.03）。总体而言，71 例（43.0%）患者出现至少 1 次 ICD 并发症，包括 42 例（25.5%）患者出现不适当电击和 36 例（21.8%）患者出现导联功能障碍。在 61 例（37.0%）一级预防患者中，分别有 0、1、2 或≥3 项指南推荐的危险因素的患者，其 ICD 治疗的年发生率分别为 4.1%、5.3%、9.5%和 13.3%。QRS 碎裂是适当 ICD 治疗的唯一独立预测因子（危险比，3.47[95%CI，1.19-10.11]），将其与当前标准相结合，可将 5 年时间依赖性曲线下面积从 0.68 提高至 0.81（=0.006）。充血性心力衰竭或左心室射血分数降低的患者非心律失常性死亡或心脏移植的风险更高（危险比，11.01[95%CI，2.96-40.95]）。
患有法洛四联症和 ICD 的患者经历了较高的适当治疗率，包括在一级预防中植入的 ICD。然而，ICD 相关并发症的长期负担相当大，这突显了需要仔细选择候选者。包括 QRS 碎裂在内的易于使用的标准组合可能会改善风险分层。注册：网址：https://www.clinicaltrials.gov；唯一标识符：NCT03837574。
