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Long-Term Outcomes and Risk Factors for Mortality After Single Ventricle Palliation for Asplenia Syndrome
Yasuyuki Kobayashi1, Shunji Sano2, Yuhei Tokuda1
1Department of Cardiovascular Surgery Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences and Okayama University Hospital Okayama Japan.
Journal of the American Heart Association
|January 30, 2026
Summary
Survival after single ventricle palliation for asplenia syndrome is suboptimal, especially with combined total anomalous pulmonary vein connection (TAPVC), pulmonary atresia (PA), and atrioventricular valve regurgitation. These factors significantly increase mortality risk.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Outcomes
Background:
- Asplenia syndrome presents complex congenital heart defects requiring single ventricle palliation.
- Long-term outcomes for this patient group remain a significant clinical challenge.
Purpose of the Study:
- To evaluate long-term survival after single ventricle palliation in patients with asplenia syndrome.
- To identify specific risk factors associated with increased mortality in this cohort.
Main Methods:
- Retrospective analysis of 151 patients with asplenia syndrome undergoing single ventricle palliation (1990-2024).
- Primary endpoint: mortality. Risk factors assessed using Cox regression over a mean 9.3-year follow-up.
Main Results:
- Overall mortality was 41%. Survival at 20 years was 53.7%.
- Total anomalous pulmonary vein connection (TAPVC), pulmonary atresia (PA), and moderate/severe atrioventricular valve regurgitation were independent mortality predictors.
- Patients with two or more risk factors had significantly lower survival (32.8% at 15 years) compared to those with none or one (72.9%).
Conclusions:
- Single ventricle palliation for asplenia syndrome has suboptimal long-term survival.
- The combination of TAPVC, PA, and atrioventricular valve regurgitation poses the greatest mortality risk.
- Novel strategies are needed to improve outcomes for this complex patient population.
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