Early Neurodevelopmental Outcomes in Patients Who Undergo Hybrid Stage I Palliation Compared to Norwood Stage I Palliation for Hypoplastic Left Heart Syndrome.

Abstract

Children with congenital heart disease are at increased risk of neurodevelopmental impairment and those with hypoplastic left heart syndrome (HLHS) are among the highest risk group. The first stage of palliation for HLHS, typically performed in the newborn period, is either a Norwood stage I procedure (NS1P) or hybrid stage 1 procedure (HS1P). Our study sought to evaluate the neurodevelopmental outcomes of patients who undergo HS1P compared to NS1P using multicenter registry data. The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry was used to identify infants who had either NS1P or HS1P and completed the Ages and Stages Questionnaires-3 (ASQ-3) at age 6 months. Patient and clinical characteristics and ASQ-3 results were compared between HS1P and NS1P groups. A 6-month ASQ-3 was completed in 459 patients, 42 patients following HS1P and 417 following NS1P. Patients who underwent HS1P were more likely to have a birth weight less than 2.5 kg (14.6% vs. 4.2%, p = 0.01) and have a genetic or chromosomal anomaly (19% vs. 8.2%, p = 0.04). Gross motor skills were the most impaired of the measured domains for the entire cohort. There were no significant differences in impairment in any ASQ-3 domain between the groups, even when the analysis was adjusted for pre-operative mechanical ventilation, non-cardiac anomaly, and center. Despite patients who undergo HS1P representing a heterogenous group with other medical comorbidities, their early neurodevelopmental outcomes were comparable to patients who underwent NS1P.