Mortality Up 25-Fold for Congenital Heart Disease With Down Syndrome

THURSDAY, Jan. 25, 2024 (HealthDay News) -- Patients with congenital heart disease (CHD) with Down syndrome (DS) have a 25-fold higher mortality rate than individuals without CHD or DS, according to a study published online Jan. 12 in the Journal of the American Heart Association.

Stella Engsner, M.D., from the University of Gothenburg in Sweden, and colleagues estimated survival in patients with CHD with versus without DS compared to matched controls without CHD or DS using linked data from Swedish health registries. Each patient with CHD was matched with eight controls without CHD or DS by sex and birth year. Mortality risk was estimated using a Cox proportional regression model.

A total of 3,285 patients with CHD-DS, 64,529 with CHD without DS, and 26,128 matched controls were identified. The researchers found that for CHD-DS versus controls, the mortality risk was 25.1 times higher, while for patients with CHD with versus without DS, the mortality rate was two times higher. In patients with CHD-DS versus controls, mortality risk was lower during more recent birth periods (hazard ratios, 46.8 and 17.7 for those born between 1970 and 1989 and between 1990 and 2017, respectively).

"Patients with CHD‐DS still have an increased risk of mortality compared with the general population and patients with CHD without DS," the authors write. "Future studies are needed to determine the underlying causes for this excess risk and evaluate whether there is a need for better follow‐up for patients with CHD‐DS."

Abstract/Full Text