Wolfe Natasha K, Schiff Mary D, Olivieri Laura J, Christopher Adam B, Fogel Mark, Slesnick Timothy C, Krishnamurthy Rajesh, Muthurangu Vivek, Dorfman Adam L, Lam Christopher Z, Weigand Justin, Robinson Joshua D, Rathod Rahul H, Alsaied Tarek
Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
J Am Coll Cardiol. 2024 Dec 17;84(25):2417-2426. doi: 10.1016/j.jacc.2024.08.063. Epub 2024 Oct 23.
Among patients with congenital heart disease, those with single ventricles have the highest risk of early mortality. Sudden cardiac death is an important cause of death in this population. Understanding the risk factors for sudden cardiac events (SCE) in Fontan patients could improve prediction and prevention.
The goal of this study was to determine the prevalence of SCE and risk factors for SCE in the Fontan population.
The Fontan Outcomes Registry Using CMR Examinations (FORCE) is an international registry collecting clinical and imaging data on Fontan patients. SCE was defined as: 1) cardiac arrest from a shockable rhythm; 2) need for emergent cardioversion/defibrillation; or 3) documented sustained ventricular tachycardia. Univariate and multivariate Cox proportional hazards regression models estimated hazard ratios for predictors of SCE.
Our sample included 3,132 patients (41% female). The median age at first cardiac magnetic resonance was 14.6 years. SCE was experienced by 3.5% (n = 109) over a median follow-up time of 4.00 years. Of the 109 patients with SCE, 39 (36%) died. On multivariable analysis, NYHA functional class >II (HR: 4.91; P < 0.0001), history of protein-losing enteropathy/plastic bronchitis (HR: 2.37; P = 0.0082), single-ventricle end-diastolic volume index >104 mL/m (HR: 3.15; P < 0.0001), and ejection fraction <50% (HR: 1.73; P = 0.0437) were associated with SCE. Kaplan-Meier analysis demonstrated that in patients with none of the above risk factors, the 4-year freedom from SCE was 99.5%.
SCE occurred in 3.5% of the study population, and one-third of patients who experienced SCE died. Mild ventricular dysfunction and dilatation by cardiac magnetic resonance, NYHA functional class, and history of protein-losing enteropathy/plastic bronchitis were associated with SCE.
在先天性心脏病患者中，单心室患者早期死亡风险最高。心源性猝死是该人群的重要死因。了解Fontan手术患者心源性猝死事件（SCE）的危险因素有助于改善预测和预防。
本研究旨在确定Fontan手术人群中SCE的发生率及其危险因素。
使用CMR检查的Fontan手术结局注册研究（FORCE）是一项收集Fontan手术患者临床和影像数据的国际注册研究。SCE定义为：1）可电击心律导致的心脏骤停；2）需要紧急心脏复律/除颤；或3）记录到的持续性室性心动过速。单因素和多因素Cox比例风险回归模型估计SCE预测因素的风险比。
我们的样本包括3132例患者（41%为女性）。首次心脏磁共振成像时的中位年龄为14.6岁。在中位随访时间4.00年期间，3.5%（n = 109）的患者发生了SCE。在109例发生SCE的患者中，39例（36%）死亡。多因素分析显示，纽约心脏协会（NYHA）功能分级>II级（HR：4.91；P < 0.0001）、蛋白丢失性肠病/塑料支气管炎病史（HR：2.37；P = 0.0082）、单心室舒张末期容积指数>104 mL/m（HR：3.15；P < 0.0001）以及射血分数<50%（HR：1.73；P = 0.0437）与SCE相关。Kaplan-Meier分析表明，在无上述任何危险因素的患者中，4年无SCE的概率为99.5%。
3.5%的研究人群发生了SCE，发生SCE的患者中有三分之一死亡。心脏磁共振成像显示的轻度心室功能障碍和扩张、NYHA功能分级以及蛋白丢失性肠病/塑料支气管炎病史与SCE相关。
