Mid-Term Outcomes of Patients With Hypoplastic Left Heart Syndrome and Left Ventricle-Coronary Artery Fistula

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiovascular Surgery

Background:

• Hypoplastic left heart syndrome (HLHS) with mitral stenosis/aortic atresia (MS/AA) presents significant challenges in infant cardiac care.
• Left ventricle-coronary artery (LV-CA) fistula is a suspected contributor to poorer outcomes in HLHS (MS/AA).

Purpose of the Study:

• To investigate the impact of LV-CA fistula on transplant-free survival in infants with HLHS (MS/AA).
• To identify factors associated with adverse outcomes in this high-risk pediatric population.

Main Methods:

• Retrospective review of 90 infants with HLHS (MS/AA) managed from birth (2008-2023).
• Comparison of outcomes between patients with and without LV-CA fistula, defined angiographically.
• Analysis of surgical palliation strategies (Surgical Stage 1 Palliation vs. Hybrid S1P) and other clinical factors.

Main Results:

• 64% of patients had an LV-CA fistula; hybrid S1P was more common in this group (36% vs 6%).
• One-year transplant-free survival was 63% with fistula versus 78% without.
• Lower gestational age, lower birth weight, initial hybrid S1P, and perioperative ECMO were significantly associated with increased risk of death or transplant (p < 0.001).
• LV-CA fistula did not reach statistical significance as an independent predictor of death or transplant (HR 1.83, p = 0.10).

Conclusions:

• Mortality remains high for infants with HLHS (MS/AA).
• While a trend towards worse survival was observed in patients with LV-CA fistula, other factors like gestational age, birth weight, and need for ECMO appear to be more significant predictors of adverse outcomes.