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

Autor: Dib N; Montreal Heart Institute, Université de Montréal Quebec Canada., Poirier N; Montreal Heart Institute, Université de Montréal Quebec Canada., Samuel M; Montreal Heart Institute, Université de Montréal Quebec Canada., Hermann Honfo S; Montreal Heart Institute, Université de Montréal Quebec Canada., Zaidi A; Nationwide Children's Hospital, Ohio State University Columbus OH., Opotowsky AR; Boston Adult Congenital Heart Service, Boston Children's Hospital and Brigham and Women's Hospital Harvard Medical School Boston MA.; Adult Congenital Heart Disease Program, Heart Institute, Cincinnati Children's Hospital Medical Center University of Cincinnati College of Medicine Cincinnati OH., Mongeon FP; Montreal Heart Institute, Université de Montréal Quebec Canada., Mondésert B; Montreal Heart Institute, Université de Montréal Quebec Canada., Kay J; University of Colorado Denver Aurora CO., Ibrahim R; Montreal Heart Institute, Université de Montréal Quebec Canada., Hamilton RM; The Hospital for Sick Children University of Toronto Toronto Canada., Fournier A; Hôpital Sainte-Justine Université de Montréal Montreal Canada., Jameson SM; Stanford University Palo Alto CA., Dore A; Montreal Heart Institute, Université de Montréal Quebec Canada., Cook S; Children's Hospital of Pittsburgh Pittsburgh PA., Cohen S; The Wisconsin Adult Congenital Heart (WAtCH) Program Medical College of Wisconsin Milwaukee WI., Chaix MA; Montreal Heart Institute, Université de Montréal Quebec Canada., Broberg CS; Oregon Health and Science University Portland OR., Aboulhosn J; University of California Los Angeles CA., Khairy P; Montreal Heart Institute, Université de Montréal Quebec Canada.
Jazyk: angličtina
Zdroj: Journal of the American Heart Association [J Am Heart Assoc] 2024 Dec 03; Vol. 13 (23), pp. e034757. Date of Electronic Publication: 2024 Nov 27.
DOI: 10.1161/JAHA.124.034757
Abstrakt: Background: 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.
Methods and Results: 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 ( P <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], P <0.001). Moreover, HLHS was associated with significantly higher rates of all components of the primary outcome.
Conclusions: Among patients with a uRV and Fontan palliation, HLHS is associated with a significantly higher rate of adverse cardiovascular events.
Databáze: MEDLINE
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