A total of 132 consecutive patients undergoing repair of partial atrioventricular septal defect were studied for mortality, left atrioventricular valve function, reoperations, left ventricular outflow tract obstruction, and supraventricular arrhythmias. Age was 2.5 months to 43 years (median, 54 months); 13 (9.8 % ) were more than 20 years old. Preoperatively, 26.5 % patients were in New York Heart Association class III/IV, 15.9 % had supraventricular arrhythmias, 25.7 % had pulmonary artery hypertension, 16.6 % had moderate-to-severe left valvular regurgitation, and 29.5 % had additional left atrioventricular valvular malformations. Autologous pericardium (n = 127) and right atrial patch (n = 5) were used to patch the defect. Left atrioventricular valvuloplasty was performed in 91 % of patients despite older age and additional malformations of the left atrioventricular valve.
Operative and late mortalities were 4.5 % and 3.2 % , respectively. Postoperative supraventricular arrhythmias were observed in 11.3 % of patients. Reoperations were required in 5.8 % patients because of a residual atrial septal defect (n = 1) and severe left atrioventricular valvular regurgitation (n = 6). At a mean follow-up of 106.82 ±55.04 months, actuarial survival was 83.70 % ± 0.07 % . The risk of death was 38.92 (95 % confidence intervals: 7.8–195.1) and 6.88 (95 % confidence intervals: 1.79–38.18) times higher in patients with grossly malformed left atrioventricular valve and preoperative pulmonary artery hypertension, respectively, by logistic regression analysis.
Detailed assessment of the valve morphology and individualized valvuloplasty techniques improves the long-term survival after repair of partial atrioventricular septal defects. The presence of grossly malformed left valvular apparatus, pulmonary artery hypertension, and moderate-to-severe left atrioventricular valve regurgitation are independent predictors of death and defect-related morbidity after surgical repair.