Association Between G6PD Deficiency and Congenital Heart Disease Incidence and Hospital Outcomes.

G6PD deficiency classically presents with hemolytic anemia, but associations have been described with atherogenesis and coronary artery disease secondary to increased oxidative stress. There are limited data that also suggest a link between G6PD deficiency and the development of congenital heart disease (CHD), possibly through the same mechanism. We hypothesize a higher incidence of CHD among individuals with G6PD deficiency as well as worse hospital outcomes for those with CHD and G6PD deficiency. We performed a retrospective review of a national administrative database from 10/2019 to 3/2024 for admissions of individuals aged 0-25 years with ICD-10 codes for moderate-to-severe CHD with and without G6PD deficiency (D55.0, D75.A). Additional data included demographics, hospital outcomes, and costs. Hospital outcomes were compared. There were 213,708 admissions with CHD and <1% had G6PD deficiency. Of the 6,363 admissions with G6PD, 289 (4.5%) had CHD and of 11,179,670 without G6PD, 213,419 (1.9%) had CHD (p < 0.001). Admissions with CHD and G6PD deficiency were predominantly Black/African American and male (consistent with G6PD deficiency epidemiology), with a longer length of stay and higher hospital costs than those without G6PD deficiency. Our findings support a higher incidence of CHD associated with G6PD deficiency as well as worse hospital outcomes for those with CHD and comorbid G6PD deficiency. More work is needed to identify a potential causative link to further understand mechanisms for CHD development.