Hypoplastic Left Heart Syndrome in U.S. Children: A 20-Year Look at Lives Lost and Gaps That Remain

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect, characterized by hypoplasia of the left ventricle, the ascending aorta, and the aortic arch often with accompanying aortic and mitral valvular anomalies. If left untreated, HLHS is uniformly fatal in infancy, yet mortality trends remain unexplored. We aim to assess trends in HLHS-related mortality among the U.S. pediatric population. Death certificate data was analyzed from the CDC WONDER database for HLHS-related mortality from 1999 to 2020. Data were stratified by year, age, sex, race, urbanization status, state and census region. Age adjusted mortality rates (AAMR) and crude mortality rates (CMR) per 1,000,000 persons and annual percent changes (APC), were calculated. Between 1999 and 2020, 7053 HLHS-related deaths occurred among the pediatric population aged < 15 years. The overall AAMRs declined steadily from 1999 to 2020 (APC: - 2.54). Males consistently had higher AAMRs than females. Non-Hispanic (NH) Blacks had the highest AAMR in 2020 (4.96), followed by Hispanic/Latinos (3.78) and NH Whites (3.47). AAMRs varied between different regions (overall AAMR Northeast (3.95), Midwest (5.70), South (5.60), and West (5.09)). Urban areas (5.07) had lower HLHS-related AAMR than rural areas (5.93). States in the top 90th percentile of HLHS-related AAMRs (West Virginia, Utah, Iowa, Arkansas, and Maine) had approximately double the AAMRs than those in the bottom-10th percentile. The majority deaths occurred in infants < 1-year (CMR: 71.09), with mortality declining steeply thereafter and minimal CMRs (≤ 0.32) in children aged 9 to 14 years. HLHS-related mortality has declined steadily from 1999 to 2020. The highest AAMRs were observed in Black males and among children living in rural areas. Targeted strategies and public health initiatives are needed to further reduce the burden of HLHS-related mortality among the high-risk groups.