Cardiac MRI Predictors of Arrhythmic Sudden Cardiac Events in Patients With Fontan Circulation

Natasha K. Wolfe, Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. Electronic address: wolfena@upmc.edu.
Mary D. Schiff, Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
Laura J. Olivieri, Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
Adam B. Christopher, Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
Mark Fogel, Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
Timothy C. Slesnick, Department of Pediatrics, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
Rajesh Krishnamurthy, Department of Radiology, Nationwide Children's Hospital, Columbus, Ohio, USA.
Vivek Muthurangu, UCL Centre for Cardiovascular Imaging, Institute of Cardiovascular Science, University College London, London, United Kingdom.
Adam L. Dorfman, Department of Pediatrics, University of Michigan Medical School, Ann Arbor, Michigan, USA.
Christopher Z. Lam, Department of Diagnostic and Interventional Radiology, Hospital for Sick Children and Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada.
Justin Weigand, Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA.
Joshua D. Robinson, Department of Pediatrics, Ann & Robert H. Lurie's Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Rahul H. Rathod, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Tarek Alsaied, Heart and Vascular Institute, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. Electronic address: https://twitter.com/nkwcardiomd.

Document Type

Journal Article

Publication Date

12-17-2024

Journal

Journal of the American College of Cardiology

Volume

84

Issue

25

DOI

10.1016/j.jacc.2024.08.063

Keywords

Fontan; arrhythmia; cardiac magnetic resonance; cardioversion; congenital heart disease; sudden death

Abstract

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/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%. 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.

Department

Pediatrics

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