Long-term outcome after early repair of complete atrioventricular septal defect in young infants

Objective: The long-term outcome after repair of complete atrioventricular septal defect in young infants is still not fully understood. The objective of this study was to evaluate data after repair for complete atrioventricular septal defect over a 25-year period to assess survival and identify risk factors for left atrioventricular valve-related reoperations. Methods: A total of 304 consecutive patients underwent surgical correction for complete atrioventricular septal defect between April 1993 and October 2018. The results for young infants (aged <3 months; n = 55; mean age 1.6 +/- 0.6 months) were compared with older infants (aged>3 months; n = 249; mean age, 5.1 +/- 5.2 months). Mean follow-up was 13.2 +/- 7.8 years (median, 14.0 years; interquartile range, 7.0-20.0). The Kaplan-Meier method was used to assess overall survival and freedom from left atrioventricular valve-related reoperation. Results: Overall, 30-day mortality was 1.0% (3/304) with no difference between young and older infants (P = 1.0). Overall survival in the total population at 20year follow-up was 95.1% (+/- 1.3%). Independent risk factors for poor survival were the presence of an additional ventricular septal defect (P = .042), previous coarctation of the aorta (P<.001), persistent left superior vena cava ( P = .026), and genetic syndromes other than Trisomy 21 (P = .017). Freedom from left atrioventricular valve-related reoperation was 92.6%(+/- 1.7%) at 20 years. There was no significant difference in left atrioventricular valve-related reoperation in young infants compared with older infants (P = .084). Conclusions: Our data demonstrated that excellent long-term survival could be achieved with early repair for complete atrioventricular septal defect, and the need for reoperations due to left atrioventricular valve regurgitation was low. Primary correction in patients aged less than 3 months is, when clinically necessary, well tolerated. Palliative procedures can be avoided in the majority of patients.

Automated summary

After repair of complete atrioventricular septal defect, there was no difference in 30-day mortality between young and older infants, with an overall 20-year survival rate of 95.1% and 92.6% freedom from left atrioventricular valve-related reoperation. Risk factors for poor survival included additional ventricular septal defect, previous coarctation of the aorta, persistent left superior vena cava, and genetic syndromes other than Trisomy 21.