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Down Syndrome With Congenital Heart Malformation

Sang C. Park, MD; Robert A. Mathews, MD; James R. Zuberbuhler, MD; Richard D. Rowe, MD; William H. Neches, MD; Cora C. Lenox, MD
Am J Dis Child. 1977;131(1):29-33. doi:10.1001/archpedi.1977.02120140031003.
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• 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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