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January 1977

Down Syndrome With Congenital Heart Malformation

Author Affiliations

From the Department of Pediatrics, University of Pittsburgh School of Medicine and the Children's Hospital of Pittsburgh (Drs Park, Mathews, Zuberbuhler, Neches, and Lenox), and the Department of Pediatrics, Johns Hopkins Medical School and Johns Hopkins Hospital, Baltimore (Dr Rowe). Dr Rowe is currently with the Hospital for Sick Children, Toronto.

Am J Dis Child. 1977;131(1):29-33. doi:10.1001/archpedi.1977.02120140031003

• Two hundred fifty-one patients with Down syndrome and congenital heart disease were seen at two institutions in recent years. Diagnosis of congenital heart disease was based on clinical (41%), catheterization (38%), surgical (11%), or autopsy data (10%). The most common lesions were endocardial cushion defect (43%), ventricular septal defect (32%), secundum atrial septal defect (10%), tetralogy of Fallot (6%), and isolated patent ductus arteriosus (4%). Thirty percent had multiple cardiac defects. The most common associated lesions were patent ductus arteriosus (16%) and pulmonic stenosis (9%). Twenty-five percent of the patients underwent cardiac surgery. Mortality in the 68 patients undergoing surgery was 26% for open heart procedures and 11% for closed heart surgery. In 32% of nonsurgically treated patients with large left-toright shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.

(Am J Dis Child 131:29-33, 1977)

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