Survival in patients with congenital heart disease and down syndrome

Abstract

Background: Increasing survivorship among patients with congenital heart disease (CHD) has been reported during the past decades. However , a number of patients with CHD have an associated Down syndrome diagnosis and the long-term prognosis is still debated. Purpose: The aim of the present study was to estimate the mortality risk in CHD patients with Down syndrome compared to a matched control population without CHD. Methods: We linked data from the Swedish National Patient Register and Cause of Death Register to identify all CHD patients born between January 1970 and December 2017. Then we identified all CHD patients with Down syndrome. Each CHD patient with Down syndrome was matched for sex and birth year with eight controls without CHD or Down syndrome from the Total Population Register. A Cox proportional regression model was used to estimate the mortality risk and Kaplan Meier for survival analysis. Results: In total, 3,285 patients with CHD and Down syndrome and 26,128 matched controls were identified. During a mean follow-up of 16.0 (±12.6)