Prevalence of extracardiac birth defects and genetic conditions in hypoplastic left heart syndrome and association with hospital utilization and mortality

Abstract

Purpose: Up to 30% of patients with congenital heart disease have associated extracardiac birth defects and/or genetic conditions (BDGC), and the presence of these may influence therapies and outcomes. We aimed to evaluate the prevalence of BDGC in hypoplastic left heart syndrome (HLHS) and evaluate the associations between specific BDGC and hospital mortality and post-Stage I mortality. Method: We included patients from the Pediatric Health Information System database from 1999-2018 with an ICD9 or ICD10 code for HLHS, no codes for conflicting congenital heart disease, …