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Cardiovascular Outcomes Associated With Hypoplastic Left Heart Syndrome Versus Other Types of Single Right Ventricle:
Nabil Dib1, Nancy Poirier1, Michelle Samuel1
1Montreal Heart Institute, Université de Montréal Quebec Canada.
Insights
Patients with a univentricular heart (uRV) and hypoplastic left heart syndrome (HLHS) undergoing Fontan palliation face higher cardiovascular event risks compared to other uRV types. This highlights HLHS as a significant risk factor in this population.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Adult Congenital Heart Disease
Background:
- Univentricular heart (uRV) morphology is linked to increased adverse cardiovascular events.
- The impact of specific uRV subtypes on outcomes remains unclear.
Purpose of the Study:
- To compare cardiovascular event rates in patients with uRV and Fontan palliation, specifically comparing hypoplastic left heart syndrome (HLHS) to other uRV forms.
Main Methods:
- A retrospective cohort study involving 171 patients with uRV and Fontan palliation.
- Comparison of composite outcomes (mortality, transplantation, arrhythmias, thromboembolism) between HLHS and non-HLHS groups.
- Time-to-event analyses accounting for competing risks over a median follow-up of 10.2 years.
Main Results:
- The composite outcome incidence was 7.1 per 100 person-years in HLHS versus 2.1 in non-HLHS (P<0.0001).
- HLHS was independently associated with a 6.13-fold increased risk of the composite outcome (HR, 6.13; 95% CI, 2.92-12.69; P<0.001).
- Significantly higher rates of all individual components of the composite outcome were observed in the HLHS group.
Conclusions:
- Hypoplastic left heart syndrome (HLHS) is a significant risk factor for adverse cardiovascular events in patients with a univentricular heart (uRV) and Fontan palliation.
- These findings emphasize the need for tailored management strategies for HLHS patients within the uRV and Fontan population.
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.
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