Hypoplastic Left Heart Syndrome with Low Birth Weight or Prematurity: What is the Optimal Approach?

BACKGROUND: Hypoplastic left heart syndrome with low birth weight or prematurity comprises a high-risk population with no optimal treatment pathway. Using the Pediatric Health Information System, we compared management approaches across the United States.

METHODS: We analyzed neonates (≤30 days) with birth weight <2500 grams or gestational age <36 weeks between 2012 and 2021. Four strategies were identified: Norwood procedure, ductus arteriosus stent + pulmonary artery banding, pulmonary artery banding + prostaglandin infusion, or comfort care. Outcomes included hospital survival, discharge disposition, staged palliation completion, and one-year transplant-free survival.

RESULTS: Of 383 infants identified, 36.4% (134/383) received comfort care, 43.9% (165/383) Norwood, 12.4% (49/383) ductal stent + pulmonary artery bands, and 8.8% (34/383) pulmonary artery bands + prostaglandins. Neonates receiving comfort care had the lowest gestational age [35 weeks (31.5-37)] and birth weight [2.0kg (1.5-2.3)]; 24.6% (33/134) had chromosomal anomalies. Infants undergoing primary Norwood had the highest birth weight [2.4kg (2.2-2.5)] and gestational age [37 weeks (35-38)]. Two-thirds [66.1% (109/165)] underwent Glenn palliation versus 18.4% (9/49) receiving ductal stent + pulmonary artery band and 35.3% (12/34) with pulmonary artery band + prostaglandins. Only 11.3% (6/53) born <2kg survived to 1 year, all following Norwood. Primary Norwood yielded higher hospital and one-year transplant-free survival than hybrid strategies.

CONCLUSIONS: Comfort care is routinely provided, particularly for infants with low birth weight, gestational age, or chromosomal anomalies. Primary Norwood offered the lowest hospital and one-year mortality and highest palliation completion rates; birth weight was the most important factor determining one-year survival.