Cardiovascular disease in Down syndrome

There is a high incidence of congenital heart disease in patients with Down syndrome. The most frequent cardiac malformations are endocardial cushion defect, ventricular septal defect, patent ductus arteriosus, and atrial septal defect. The majority of these defects are hemodynamically significant and result in clinical signs and symptoms of cardiopulmonary compromise. Medical management is effective temporarily in many cases, but does not affect the development of pulmonary artery hypertension or pulmonary vascular disease. Surgical management of these patients is indicated. Improvement in surgical techniques has allowed intervention in infancy with satisfactory long-term results. The development of pulmonary artery hypertension and pulmonary vascular disease is significant in patients with Down syndrome and often affects the long-term prognosis of these children. Fortunately, improvement in the medical and surgical management of patients with serious heart defects has advanced to the point where most cardiac centers in this country are able to perform surgical procedures with an acceptable risk. Children with Down syndrome and congenital heart disease can be successfully treated early in life. Future concerns, in the light of a changing sociolegal environment, center around possible prevention, early recognition, and improved management.