Abstract
BACKGROUND: Surgical outcomes of first-stage palliation for hypoplastic left heart syndrome (HLHS) are vastly improved from prior eras and are well described, but rates and determinants of nonsurgical management are understudied, particularly on the national scale. METHODS: The Pediatric Health Information System database was used to identify all index neonatal HLHS admissions between 2015 and 2021 in the United States. Surgical palliation included cardiac surgery and transplantation during the index admission. Multivariable binary logistic regression was used to identify factors independently predictive of nonsurgical management, represented by odds ratio <1.0. RESULTS: In total, 3902 HLHS neonatal admissions to 48 tertiary care centers occurred. Eighteen percent (n = 710) of neonates did not receive surgical palliation. Ninety-seven percent of nonsurgical patients died in the hospital or were discharged to comfort care, whereas 84% of surgically palliated neonates survived to discharge. Nonsurgical patients had greater rates of prematurity, birth weight <2.5 kg, female sex, and chromosomal abnormality (all P < .01). Factors independently associated with nonoperative management included admission to a low-volume surgical program (odds ratio, 0.2 [95% CI, 0.15-0.26]), birth weight <2.5 kg (0.30 [0.24-0.37]), chromosomal abnormality (0.55 [0.43-0.71]), and non-White race/ethnicity (0.68 [0.56-0.82]). CONCLUSIONS: In the contemporary era of neonatal HLHS management, nearly one-fifth of neonates do not undergo surgical palliation in the United States. Additional work can outline the precise reasons underlying such high rates of nonoperative management in hopes of ensuring equitable care for all children with HLHS.