IS DOWN-SYNDROME A RISK FACTOR FOR POOR OUTCOME AFTER REPAIR OF CONGENITAL HEART-DEFECTS

Down syndrome is commonly associated with significant congenital heart disease with the potential for early development of pulmonary hyperte nsion. As such, children with Down syndrome may be at increased risk f or both perioperative and long-term mortality. The purpose of this stu dy, using data collected from a population-based outcomes study, is to analyze the potential role that Down syndrome plays in the outcome of surgically ''corrected'' congenital heart disease. Data were collecte d from a registry of all Oregon residents who, in the period 1958 to t he present, had a reparative operation for one of 14 congenital cardia c malformations when younger than 18 years (N = 3965 patients). Down s yndrome was present in 289 (7%) of the total registry patients. In eva luating the cardiac mortality associated with Down syndrome for each o f the repaired cardiac malformations, only complete atrioventricular s eptal defect was associated with significantly higher perioperative (1 3% vs 5%) as well as higher overall late cardiac mortality through 20 years after the operation (20% vs 5%; p = 0.04). The survival outcomes for each of the other cardiac malformations were similar for children with and without Down syndrome.