Does Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life?

BACKGROUND

Down syndrome is known to affect the natural history of complete atrioventricular septal defect. We analyzed whether Down syndrome affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life.

METHODS

Repairs of complete atrioventricular septal defect were performed in 64 infants. Thirty-four infants were associated with Down syndrome, while the other 30 were non-Down patients.

RESULTS

Complete follow-up rate was 95% with mean follow-up period of 99+/-47 months (maximum 169 months) in Down patients and 80+/-64 months (maximum 213 months) in non-Down patients. There was one operative death in each group (mortality rate of 2.9% in Down patients and 3.3% in non-Down patients), and three patients died at the late phase (one in Down patients and two in non-Down patients). Five patients underwent re-operation due to postoperative left atrioventricular valve regurgitation (one in Down patients and four in non-Down patients). Freedom from re-operation for left atrioventricular valve regurgitation and actuarial survival rate at 13 years were 96+/-4 and 94+/-4% in Down patients and 85+/-7 and 90+/-5% in non-Down patients (not significantly different).

CONCLUSIONS

Down syndrome does not affect the long-term results of complete atrioventricular septal defect when the defect is repaired during the first year of life.