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Comparative Study
. 2024 Dec 3;13(23):e034757.
doi: 10.1161/JAHA.124.034757. Epub 2024 Nov 27.

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

Nabil Dib  1 , Nancy Poirier  1 , Michelle Samuel  1 , Sewanou Hermann Honfo  1 , Ali Zaidi  2 , Alexander R Opotowsky  3   4 , François-Pierre Mongeon  1 , Blandine Mondésert  1 , Joseph Kay  5 , Reda Ibrahim  1 , Robert M Hamilton  6 , Anne Fournier  7 , Susan M Jameson  8 , Annie Dore  1 , Stephen Cook  9 , Scott Cohen  10 , Marie-A Chaix  1 , Craig S Broberg  11 , Jamil Aboulhosn  12 , Paul Khairy  1
Affiliations
Comparative Study

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

Nabil Dib et al. J Am Heart Assoc. .

Abstract

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.

Keywords: Fontan surgery; hypoplastic left heart syndrome; univentricular heart.

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Figures

Figure 1
Figure 1. Time trends for Fontan surgery in patients with HLHS and other forms of single right ventricle (non‐HLHS).
HLHS indicates hypoplastic left heart syndrome.
Figure 2
Figure 2. Freedom from atrial arrhythmia, thromboembolic event, transplantation, or death according to initial diagnosis at Fontan completion.
HLHS indicates hypoplastic left heart syndrome.
Figure 3
Figure 3. Cumulative incidence of (A) cardiac transplantation or death, (B) atrial arrhythmia, and (C) thromboembolic events according to the diagnosis of HLHS versus non‐HLHS.
HLHS indicates hypoplastic left heart syndrome.
See this image and copyright information in PMC

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