Results of surgical treatment of congenital heart defects in children withDown's syndrome

We analyzed early and late results of surgical treatment of 100 consecutive children with Down's syndrome (DS) and congenital heart defect (CHD) who w ere operated on between 1990 and 1997. Fifty had common atrioventricular ca nal (CAVC), 24 ventricular septal defect, 8 the ostium primum atrial septal defect, 8 tetralogy of Fallot (TOF), 3 patent ductus arteriosus, 3 the ost ium secundum atrial septal defect, and 4 CAVC coexisting with TOF. In 93 pa tients total correction was performed. The total death rate was 6%. Death i n the CAVC group was 8%, but it decreased to 2.7% during the past 3 years. The children who were followed up (from 7 months to 6 years; mean, 39 month s) are in NYHA class I or II. There were no reoperations, The postoperative course was complicated by pulmonary infections in 38% of patients, which c onverted to generalized infection in 10% and was the cause of death in 8% o f patients, These results indicate that CHD in DS children can be repaired with a low death rate and low incidence of severe mitral atrioventricular v alve regurgitation in the CAVC group. A high incidence of severe infections can influence the final results. Repair of CHD in infancy helps to elimina te problems connected with congestive heart failure and pulmonary hypertens ion.