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Cardiac MRI Predictors of Arrhythmic Sudden Cardiac Events in Patients With Fontan Circulation
Natasha K Wolfe1, Mary D Schiff1, Laura J Olivieri1
1Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
Insights
Sudden cardiac events (SCE) affect 3.5% of Fontan patients, with risk factors including poor functional class and heart dilation. Early identification of these factors is crucial for preventing sudden cardiac death in this high-risk group.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease Research
Background:
- Single ventricle patients face the highest mortality risk in congenital heart disease.
- Sudden cardiac death (SCD) is a significant cause of mortality in Fontan patients.
- Identifying risk factors for sudden cardiac events (SCE) can improve prediction and prevention strategies.
Purpose of the Study:
- To determine the prevalence of SCE in the Fontan population.
- To identify risk factors associated with SCE in Fontan patients.
Main Methods:
- Utilized data from the Fontan Outcomes Registry Using CMR Examinations (FORCE) international registry.
- Defined SCE as cardiac arrest (shockable rhythm), emergent cardioversion/defibrillation, or sustained ventricular tachycardia.
- Employed univariate and multivariate Cox proportional hazards regression models to analyze predictors of SCE.
Main Results:
- 3.5% of 3,132 Fontan patients experienced SCE over a median follow-up of 4 years.
- NYHA functional class >II, protein-losing enteropathy/plastic bronchitis, dilated single ventricle (end-diastolic volume index >104 mL/m²), and reduced ejection fraction (<50%) were significant risk factors for SCE.
- Patients with none of these risk factors had a 99.5% freedom from SCE at 4 years.
Conclusions:
- SCE occurred in 3.5% of the study population, with one-third of these patients dying.
- Mild ventricular dysfunction and dilation identified by cardiac magnetic resonance, along with NYHA functional class and a history of protein-losing enteropathy/plastic bronchitis, are associated with SCE in Fontan patients.
Background:
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.
Objectives:
The goal of this study was to determine the prevalence of SCE and risk factors for SCE in the Fontan population.
Methods:
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.
Results:
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/m2 (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%.
Conclusions:
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.
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