Hypoplastic Left Heart Syndrome With Low Birth Weight or Prematurity: What Is the Optimal Approach?

Area of Science:

• Pediatric Cardiology
• Neonatal Surgery
• Congenital Heart Disease Research

Background:

• Hypoplastic left heart syndrome (HLHS) in neonates with low birth weight or prematurity presents a high-risk population.
• There is a lack of an optimal treatment pathway for these vulnerable infants.
• Management strategies across the United States were compared to identify best practices.

Purpose of the Study:

• To compare the effectiveness of different management strategies for HLHS in neonates with low birth weight or prematurity.
• To identify factors influencing survival and treatment outcomes in this high-risk population.

Main Methods:

• Analysis of neonates (≤30 days) with birth weight <2500 grams or gestational age <36 weeks from 2012-2021 using the Pediatric Health Information System.
• Comparison of four management strategies: Norwood procedure, ductus arteriosus stent + pulmonary artery banding, pulmonary artery banding + prostaglandin infusion, or comfort care.
• Outcomes assessed included hospital survival, discharge disposition, staged palliation completion, and 1-year transplant-free survival.

Main Results:

• The Norwood procedure was performed in 43.9% of infants, comfort care in 36.4%, ductal stent + pulmonary artery banding in 12.4%, and pulmonary artery banding + prostaglandins in 8.8%.
• Infants receiving comfort care had lower gestational age and birth weight, with 24.6% having chromosomal anomalies.
• Primary Norwood procedure resulted in higher hospital survival and 1-year transplant-free survival compared to hybrid strategies. Birth weight <2 kg was associated with only 11.3% 1-year survival, all post-Norwood.

Conclusions:

• Comfort care is frequently used for infants with low birth weight, prematurity, or chromosomal anomalies.
• The primary Norwood procedure demonstrated the lowest hospital mortality and 1-year mortality, alongside the highest palliation completion rates.
• Infant birth weight was identified as the most critical determinant of 1-year survival in this cohort.