Down syndrome with congenital heart malformation.
Two hundred fifty-one patients with Down syndrome and 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 uncerwent cardiac surgery. Motality 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-to-right shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.
D000015:Abnormalities, Multiple / Q000175:diagnosis; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications*; D004374:Ductus Arteriosus, Patent / Q000150:complications; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000401:mortality / Q000601:surgery; D006343:Heart Septal Defects / Q000150:complications; D006344:Heart Septal Defects, Atrial / Q000150:complications; D006345:Heart Septal Defects, Ventricular / Q000150:complications; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D013771:Tetralogy of Fallot / Q000150:complications