Racial/Ethnic Disparities In Age At Death During Early Childhood Among Children with Congenital Heart Defects

Infants with congenital heart defects (CHD) have increased risk of childhood morbidity and mortality.  Racial/ethnic disparities in age at death during infancy are well documented.   However, less is known about racial/ethnic disparities in age of death during early childhood for specific CHD phenotypes.  Texas Birth Defect Registry data were used in a retrospective cohort study of 19,406 singleton, live-born infants, diagnosed with a CHD and born between 1/1/1996 and 12/31/2003 to non-Hispanic (NH) white, NH-black, or Hispanic women.  Registry data were linked to death records to ascertain deaths through 12/31/2005.  Kaplan-Meier survival estimates were computed and hazard ratios (HR) and 95% confidence intervals (CI) were calculated from multivariable Cox-proportional hazard regression models to determine the adjusted effect of maternal race/ethnicity on mortality for each CHD phenotype during the neonatal, post-neonatal and childhood periods.  Racial/ethnic disparities in age at death were most pronounced during the post-neonatal period and persisted into early childhood.  NH-Blacks and Hispanics with transposition of the great arteries (HR=4.15; 95% CI: 2.33, 7.37 and HR=1.82; 95% CI: 1.11, 2.98, respectively) pulmonary valve atresia/stenosis (HR=3.10; 95% CI: 1.67, 5.78 and HR=1.88; 95% CI: 1.09, 3.23, respectively) had increased risk of death during early childhood compared to NH-Whites.  NH-Blacks with ventricular septal defect (HR=1.82, 95% CI: 1.26, 2.62) and atrial septal defect (HR=1.58; 95% CI: 1.15, 2.18) and Hispanics with tetralogy of Fallot (HR=2.21; 95% CI: 1.10, 4.45) and atrioventricular septal defect (HR=1.75; 95% CI: 1.08, 2.84) also had increased risk of mortality during early childhood compared to NH-Whites. Racial/ethnic disparities in age at death in early childhood for specific CHD phenotypes are present but of unknown etiology.  Elucidation of factors associated with early childhood CHD mortality will aid in development of public health and clinical strategies to reduce racial/ethnic disparities in childhood mortality.