Surgical management of congenital heart disease in Down's syndrome

Children with Down's syndrome have been thought to be at increased risk for operative treatment of congenital heart disease (CHD), but this impression has not been objectively studied. The hospital course of 60 Down's patients with CHD treated operatively between 1953 and 1975 was analyzed. Thirty three boys and 27 girls (2 mth to 18 yr of age) had 1 or more cardiac surgical procedures, 26 as infants (<2 yr) and 34 as children (2 to 18 yr). Clinical severity of the cardiac condition was graded objectively in all patients preoperatively and postoperatively from 0 (asymptomatic) to 4+ (severe cyanosis or uncontrolled congestive heart failure). Heart lesions ranked in incidence as follows: atrioventricular canal (AVC), 40%; ventricular septal defect (VSD), 27%; patent ductus arteriosus (PDA), 12%; tetralogy of Fallot (TOF), 8%; primum atrial septal defect (ASD), 6%; secundum ASD, 5%, and tricuspid atresia, 2%. Eleven of 24 AVC patients died and 13 improved in clinical severity. Two of 16 patients died and 13 improved in clinical severity. Two of 16 patients with VSD died, 1 deteriorated, 1 was clinically unchanged, and 12 improved. Five of 7 PDA patients were improved and 2 remained asymptomatic. Three of 5 TOF patients died and 2 improved. All 4 patients with primum ASD survived and improved; 1 of 3 with secundum ASD died and 2 improved. One patient with tricuspid atresia died. The number of hospitalizations was reduced from an average of 3.0 per patient preoperatively to 0.6 postoperatively in survivors. The authors conclude from this series, and from lesion by lesion comparison with the CHD surgery literature, that Down's syndrome patients with CHD can undergo cardiac surgery with mortality rates comparable to those in the non Down's population, and with impressive postoperative improvement. The operative mortality rate for TOF was particularly high, but surgery may be necessitated by the patient's severe condition.