Anatomical Ventricular Type Determines 30-Year Outcomes in Fontan Patients: Insights from National Korean Registry

초록

Background: Systemic right ventricular morphology is associated with poor Fontan outcomes, but its independent effect remains uncertain due to confounding by atrioventricular valve regurgitation (AVVR). The prognostic role of two adequately sized ventricles is also unclear. This study aimed to evaluate the independent impact of ventricular morphology on long-term Fontan outcomes after adjustment for AVVR, using a large national registry. Methods: A total of 1631 Fontan survivors were analyzed. Death or heart transplantation and Fontan failure were primarily evaluated using inverse probability of treatment weighting. Fontan failure was defined as death, heart transplantation, Fontan takedown or conversion, protein-losing enteropathy, plastic bronchitis, or New York Heart Association class III/IV. Results: The cohort included 35.8% systemic right ventricular morphology, 38.6% systemic left ventricular morphology, and 25.6% two adequately sized ventricular morphology. Systemic right ventricular morphology was associated with a higher risk of death or heart transplantation compared with systemic left ventricular morphology (HR, 1.699; 95% CI, 1.130-2.554; p = 0.011). A similar pattern was observed for Fontan failure (HR, 1.487; 95% CI, 1.084-2.038; p = 0.014). In comparison, two adequately sized ventricular morphology did not demonstrate a clear advantage over systemic left ventricular morphology for death or heart transplantation (HR, 1.582; 95% CI, 0.977-2.563; p = 0.062) or Fontan failure (HR, 1.207; 95% CI, 0.815-1.788; p = 0.347), and showed no significant difference compared with systemic right ventricular morphology. Conclusion: Systemic right ventricle morphology was associated with the poorest long-term outcomes after adjustment for AVVR. Despite having two adequately sized ventricles, this morphology group showed no advantage over systemic left ventricular morphology and did not differ significantly from systemic right ventricular morphology.

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