Population-Based Perspective of Long-Term Outcomes After Surgical Repair of Partial Atrioventricular Septal Defect

Background This investigation was designed to determine long-term survival, reoperation rates, and functional status after surgical repair of partial atrioventricular septal defect (PAVSD). Methods This population-based cohort study with cumulative, prospective follow-up by questionnaire and medical record review included all patients aged younger than 19 years old in the state of Oregon who underwent surgical repair of a PAVSD from 1958 to 2000. The incidence of early death, late death, and reoperation for left atrioventricular valve pathology were determined. Patient-reported health status as measured by the Medical Outcomes Study Short Form 12 (SF-12) was obtained for patients without Down syndrome when they were aged older than 15 years. Results Repair of PAVSD was done in 133 patients. Median follow-up was 8.7 years for a total of 1541 person-years. Mean age at the initial operation was 5.2 ± 5.1 years (median, 3.4 years). Mean weight was 19.2 ± 16.0 kg (median, 13.2 kg). Survival was 95% at 30 days, 87% at 10 years, and 78% at 30 years. Reoperation for left atrioventricular valve pathology was done 15 patients (11.3%). Lower weight, absence of Down syndrome, and lack of mitral valve cleft repair were significantly associated with undergoing reoperation. Patient-reported health status was obtained in 35 patients. For this group, the mean SF-12 summary scores for the physical component (52.8 ± 9.0) and the mean mental component (50.3 ± 11.0) were not significantly different from age-adjusted norms. Conclusions The survival rate for this simple cardiac defect is lower than the general population. In addition, the reoperation rate is significant. Despite this, in general, patients without Down syndrome can expect normal functional health status.