Abstract 10135: Mortality From Birth to Young Adulthood Among Individuals With Congenital Heart Defects: Results From the Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being

Introduction: Limited information exists on long-term mortality of individuals with congenital heart defects (CHD) in the United States. Our objective was to evaluate mortality and related factors among a population-based sample of individuals with CHD born in three U.S. locations. Methods: Birth defects surveillance system data on individuals with CHD born between 1980-1997 in Arkansas, Arizona, and Atlanta, Georgia, for the Congenital Heart Survey to Recognize Outcomes, Needs, and well-beinG (CH STRONG) were linked to death records through 2015. Survival probability was estimated using Kaplan-Meier survival curves. For those who survived past one year of age, associations between age at death up to 35 years and characteristics at birth were assessed by adjusted hazards ratios and 95% confidence intervals (CIs) from multivariable Cox proportional hazards models. Standardized mortality ratios (SMRs) and CIs comparing observed to expected deaths based on age-, sex-, year-, race/ethnicity-, and site-specific population mortality rates from the National Vital Statistics System (NVSS) data were calculated. Results: Of 11,333 included individuals from CH STRONG, 9,630 (85%) survived to one year of age; of those, probability of survival to age 35 years was 95.8%. Hazard ratios for death up to age 35 years were elevated for those with severe defects, non-cardiac congenital anomalies, and maternal race other than non-Hispanic (NH) white (Figure). The SMR comparing CH STRONG to NVSS was 4.6 (CI: 4.3, 5.0); SMRs remained elevated when limiting to NH white (SMR=4.1, CI: 3.7, 4.5), NH Black (SMR=4.6, CI: 3.9, 5.4), males (SMR=3.6, CI: 3.3, 4.1) and females (SMR=6.8, CI: 6.0, 7.6). Conclusions: Individuals with CHD who survived to 1 year of age had 95.8% survival to age 35 years, with their risk of mortality 4.6 times that of the general population. People with severe defects, non-cardiac congenital anomalies, and maternal race other than NH white had the greatest mortality risk.