Late functional outcomes after repair of tetralogy of Fallot with atrioventricular septal defect: a double case-match control study.

OBJECTIVES: We sought to elucidate late functional outcomes of the right ventricular outflow tract and atrioventricular valves after repair of tetralogy of Fallot with atrioventricular septal defect.

METHODS: From 1990 to 2010, the data from 41 patients who underwent repair were retrospectively reviewed. The median age at repair was 22.4 months (interquartile range, 10.8-41.6 months). Of the 41 patients, 13 (32%) had received previous palliations. The preoperative anatomic differences, physiologic differences, and long-term functional outcomes were determined using a double case-matched control with isolated tetralogy of Fallot and isolated atrioventricular septal defect.

RESULTS: The right ventricular outflow tract was reconstructed, with the pulmonary valve preserved in 23 patients (56%). There were 3 early deaths and no late deaths. Survival was 92.1% at 15 years. During a median follow-up period of 5.9 years (interquartile range, 0.14-13.7 years), 29 reinterventions were performed in 13 (32%) patients. Freedom from all reintervention at 15 years was 52.8%. Of the 29 procedures, 12 (41%) were related to the right ventricular outflow tract. Freedom from right ventricular outflow tract-related reintervention was greater in patients who had their pulmonary valve preserved (95% vs 70% at 10 years; P = .046). Reinterventions were performed for the atrioventricular valve in 2 (5%) patients and subaortic stenosis in 1 (2%) patient. Freedom from right ventricular outflow tract-related reintervention (tetralogy of Fallot with atrioventricular septal defect, 88.6% vs tetralogy of Fallot alone, 83.9% at 5 years; P = .809) and atrioventricular valve/left ventricular outflow tract-related reintervention (tetralogy of Fallot with atrioventricular septal defect, 95.2% vs atrioventricular septal defect alone, 86.0% at 5 years; P = .332) were comparable between the tetralogy of Fallot with atrioventricular septal defect and matched control groups.

CONCLUSIONS: Late survival and atrioventricular valve function after repair of tetralogy of Fallot with atrioventricular septal defect were excellent. Pulmonary valve preservation and avoidance of an artificial conduit were associated with greater freedom from right ventricular outflow tract reintervention. In the current era, the surgically modified history of tetralogy of Fallot with atrioventricular septal defect is not significantly different from that of isolated tetralogy of Fallot or isolated atrioventricular septal defect.