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Cardiovascular Outcomes Associated With Hypoplastic Left Heart Syndrome Versus Other Types of Single Right Ventricle: A Multicenter Study
Journal article   Open access   Peer reviewed

Cardiovascular Outcomes Associated With Hypoplastic Left Heart Syndrome Versus Other Types of Single Right Ventricle: A Multicenter Study

Nabil Dib, Nancy Poirier, Michelle Samuel, Sewanou Hermann Honfo, Ali Zaidi, Alexander R Opotowsky, François-Pierre Mongeon, Blandine Mondésert, Joseph Kay, Reda Ibrahim, …
Journal of the American Heart Association, v 13(23), e034757
03 Dec 2024
DOI: https://doi.org/10.1161/JAHA.124.034757
PMID: 39604028
Featured in Collection :   UN Sustainable Development Goals @ Drexel
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https://doi.org/10.1161/JAHA.124.034757View
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Abstract

Adolescent Arrhythmias, Cardiac - epidemiology Arrhythmias, Cardiac - etiology Child Child, Preschool Female Fontan Procedure - adverse effects Heart Transplantation Heart Ventricles - abnormalities Heart Ventricles - diagnostic imaging Heart Ventricles - physiopathology Humans Hypoplastic Left Heart Syndrome - complications Hypoplastic Left Heart Syndrome - mortality Hypoplastic Left Heart Syndrome - surgery Incidence Infant Male North America - epidemiology Retrospective Studies Risk Factors Thromboembolism - epidemiology Thromboembolism - etiology Time Factors Treatment Outcome Univentricular Heart - complications Univentricular Heart - mortality Univentricular Heart - surgery Palliative Care
The univentricular heart with a predominant right ventricle morphology (uRV) has been associated with a higher rate of adverse cardiovascular events. It remains to be determined whether the specific type of uRV influences outcomes. A North American multicenter retrospective cohort study was conducted by the Alliance for Adult Research in Congenital Cardiology on individuals with a uRV and total cavopulmonary connection Fontan. The incidence of a composite outcome consisting of all-cause mortality, cardiac transplantation, atrial arrhythmias, or thromboembolic events was compared among patients with Fontan palliation who had hypoplastic left heart syndrome (HLHS) versus other forms of uRV (non-HLHS). All components of the composite outcome were classified by a blinded adjudicating committee. Competing risks were taken into account in time-to-event analyses. A total of 171 patients with uRV of whom 76 (44.4%) had HLHS were followed for 10.2±5.7 years. The composite outcome occurred in 7.1 versus 2.1 cases per 100 person-years in patients with HLHS versus non-HLHS ( <0.0001). In multivariable analyses, HLHS was associated with a significantly higher risk of the composite outcome (hazard ratio [HR], 6.13 [95% CI, 2.92-12.69], <0.001). Moreover, HLHS was associated with significantly higher rates of all components of the primary outcome. Among patients with a uRV and Fontan palliation, HLHS is associated with a significantly higher rate of adverse cardiovascular events.

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Web of Science research areas
Cardiac & Cardiovascular Systems
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