Surgical Outcomes in Children With Partial and Transitional Atrioventricular Septal Defect.

BACKGROUND: This study aimed to evaluate the long-term outcomes of partial and transitional atrioventricular septal defect repair, focusing on left atrioventricular valve reoperation. METHODS: We conducted a retrospective review of 104 children who underwent partial or transitional atrioventricular septal defect repair between August 1983 and January 2021. The major outcomes included patient survival and left atrioventricular valve reoperation. RESULTS: Median age at repair was 4.0 (0.1-17.0) years, with 17 patients being infants (age ≤ 1 year; 16.3%). All but eight patients (92.3%) underwent left atrioventricular valve cleft closure. After initial repair, there were 18 cases of moderate-to-severe left atrioventricular valve regurgitation (17.3%). Three in-hospital deaths (2.9%) and four late deaths (3.8%) occurred. At follow-up (median 14.3 years), actuarial survival was 95.1% and 93.0% at 1 and 20 years, respectively, and 16 patients (15.4%) had undergone a total of 19 left atrioventricular valve reoperations. Initial repair performed during infancy was associated with significantly higher mortality than a repair performed after infancy (35.3% vs 1.5%, P < .01, hazard ratio = 26.4). On multivariable analysis, repair during infancy was associated with mortality (P < .01, hazard ratio = 27.4, 95% confidence interval = 2.7-283). Partial or no cleft closure of left atrioventricular valve (P = .03, hazard ratio = 4.7, 95% confidence interval = 1.2-18.8) and moderate-to-severe left atrioventricular valve regurgitation after repair (P < .01, hazard ratio = 9.9, 95% confidence interval = 3.0-32.2) were associated with left atrioventricular valve reoperation. CONCLUSIONS: Partial and transitional atrioventricular septal defect repair outcomes were generally satisfactory. However, repair during infancy had worse survival outcomes, and moderate-to-severe left atrioventricular valve regurgitation after a repair was associated with future left atrioventricular valve reoperation.

Contemporary results after repair of partial and transitional atrioventricular septal defects.

OBJECTIVE: The exact incidence and risk factors for reoperation in partial and transitional atrioventricular septal defects are unclear. The goal of this study was to assess risk factors for left atrioventricular valve and left ventricular outflow tract reoperation in partial and transitional atrioventricular septal defects. METHODS: All patients undergoing partial and transitional atrioventricular septal defects repair between 1995 and 2017 were reviewed. Patients were classified as infants (<1 year), toddlers (1-3 years), children (3-17 years), and adults (≥18 years). Survival and reoperation were assessed using log-rank test and Cox models for univariate and multivariable analyses, respectively. RESULTS: Overall, 265 patients underwent partial and transitional atrioventricular septal defects repair (partial: 177 [67%]). Median age was 2 years. The cohort included 73 infants (28%), 85 toddlers (32%), 94 children (35%), and 13 adults (5%). Trisomy 21 was present in 76 patients (29%), and in 216 patients (83%), the zone of apposition was completely closed. Perioperative mortality was 0.8%. Complete heart block did not develop in any patients. Ten-year survival and freedom from reoperation were 98% and 81%, respectively. On multivariable analysis, trisomy 21 (hazard ratio [HR], 0.16) and older age compared with infants (toddlers: HR, 0.35; children: HR, 0.25) were protective for any reoperation, whereas heterotaxy (HR, 3.43) was a risk factor. For left atrioventricular valve reoperation, toddlers (HR, 0.35), children (HR, 0.25), and trisomy 21 (HR, 0.16) remained protective, whereas left atrioventricular valve anomaly was a risk factor (HR, 2.61). Likewise, for left ventricular outflow tract reoperation, toddlers (HR, 0.24) and children (HR, 0.06) were protective. CONCLUSIONS: Mortality after partial and transitional atrioventricular septal defects repair is minimal, yet reoperation for left atrioventricular valve disease and left ventricular outflow tract obstruction remains significant. Patients requiring repair during infancy are at higher risk of reoperation.