Life expectancy and social adaptation in 373 children with Down syndrome with and without congenital heart disease (CHD) were assessed retrospectively. Survival at age 24 years was 92.2% for patients without CHD (n=200), and 74.6% for those with CHD (n=173). Survival for those who underwent operation for cardiovascular lesions (n=95) was 87.8%, and for those not operated on despite hemodynamically significant cardiovascular lesions (n=39), it was 41.4%. Cardiac functional capacity was better in the children without congenital heart disease and in the group operated on, where most patients also attained good social adaptation. We conclude that children with Down syndrome with congenital heart disease should undergo early cardiac evaluation and surgery if indicated.