Long-term follow-up of partial atrioventricular septal defect repair in adults

Patients presenting with partial atrioventricular septal (PAVC) defects in adulthood are rare. Eight adult patients with this congenital heart defect who had surgical repair are described, along with their long-term postoperative follow-up data. Average age at operation was 48 years, and average follow-up was 13.4 years. There was no operative mortality. All but one patient were in NYHA functional class 1 or 2 postoperatively. Three patients had suffered late onset of progressive mitral regurgitation leading to severe congestive heart failure 15 to 25 years postoperatively. The number of adult patients with repaired PAVC defects who have eventual mitral valve deterioration may be higher than estimated in the current literature. Because of the unpredictability of mitral valve function, lifelong follow-up should be stressed in these patients.