Background: Between 1983 and 1994, 115 infants and children underwent repair of a complete atrioventricular canal defect with the two-patch technique and routine mitral valve "cleft" closure.
Methods: A retrospective review of these 115 patients was performed. Age at the time of repair ranged from 1 month to 108 months (mean age, 14.2 +/- 16.5 months; median age, 8 months). Preoperative cardiac catheterization in 113 patients revealed a mean pulmonary to systemic flow ratio of 3.37 +/- 1.8, a mean pulmonary artery systolic pressure of 71.1 +/- 15.7 mm Hg, and a mean pulmonary vascular resistance of 4.9 +/- 3.3 units. Associated anomalies included Down's syndrome (99 patients), patent ductus arteriosus (47), and coarctation of the aorta (4). Rastelli classification was A (76 patients), B (10), C (24), and unknown (5). Twenty-four patients had intraoperative epicardial or transesophageal echocardiography.
Results: Although there was a trend toward increasing mean preoperative pulmonary vascular resistance with age from 2.1 +/- 0.9 units (0 to 3 months) to 4.0 +/- 2.6 units (4 to 6 months) to 5.7 +/- 3.0 units (7 to 12 months), the mean pulmonary vascular resistance of each age group was not significantly different from that of the main group. The operative survival rate was 94% (seven early deaths) and the overall survival rate, 91% (three late deaths). Intraoperative echocardiography altered the surgical therapy for 1 patient. No patient has required reoperation for a residual ventricular septal defect. Four patients (3.5%) had heart block requiring permanent pacemakers. Eight patients (7%) required reoperation for mitral insufficiency; 6 of whom had successful repair of a residual cleft.
Conclusions: For infants with complete atrioventricular canal defect, repair using the two-patch technique with routine mitral valve cleft closure at 4 to 6 months of age results in a low operative mortality, a low incidence of permanent heart block, and a low reoperation rate for mitral insufficiency.