TY  - EJOU
AU  - Baek, Jae Suk 
AU  - Kim, Soo-Jin 
AU  - Lee, Sang-Yun 
AU  - Lee, Chang-Ha 
AU  - Park, Chun Soo 
AU  - Choi, Eun Seok 
AU  - Ko, Hoon 
AU  - An, Hyo Soon 
AU  - Kang, I. Seok 
AU  - Yoon, Ja Kyoung 
AU  - Lee, Jae-Young 
AU  - Song, Jinyoung 
AU  - Lee, Joo Won 
AU  - Huh, June 
AU  - Ahn, Kyung-Jin 
AU  - Jung, Se-Yong 
AU  - Cha, Seul Gi 
AU  - Kim, Yeo-Hyang 
AU  - Lee, Young-Seok 
AU  - Cho, Sang-Hoon 

TI  - Anatomical Ventricular Type Determines 30-Year Outcomes in Fontan Patients: Insights from National Korean Registry
T2  - Structural and Congenital Heart Disease

PY  - 2026
VL  - 21
IS  - 2
SN  - 3071-1738

AB  - 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; <i>p</i> = 0.011). A similar pattern was observed for Fontan failure (HR, 1.487; 95% CI, 1.084–2.038; <i>p</i> = 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; <i>p</i> = 0.062) or Fontan failure (HR, 1.207; 95% CI, 0.815–1.788; <i>p</i> = 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.
KW  - Fontan; ventricular morphology; outcome

DO  - 10.32604/schd.2026.077034