Objective: Since a functional right ventricle is desirable when repairing pulmonary atresia with intact ventricular septum, we sought to determine the factors that portend a successful biventricular repair in these children.
Methods: A review of operative records at UCLA between 1982 and 2001 revealed 56 patients diagnosed with pulmonary atresia with intact ventricular septum that underwent either a partial (n=26) or complete biventricular repair. Kaplan-Meier survival curves with log rank statistics were used to evaluate the influence of demographic, technical, and anatomic factors on survival and need for reoperation.
Results: Five-year actuarial survival following biventricular repair was 91.5%. Non-Caucasian race (p=0.011) and omission of palliative right ventricular outflow tract obstruction (RVOTO) relief (p=0.042) were risk factors for early death. All patients with adequate follow-up required reoperation with median duration of 6.9 years. The most common cause of early reoperation (<1 year) was wound infection (35.3%) while pulmonary valve replacement (58.8%) constituted the majority of late reoperations. Risk factors for wound infection included female sex (p=0.011) and use of a synthetic transannular patch (p=0.085). The most significant risk factor for early pulmonary valve replacement was the use of a monocusp pericardial valve in the pulmonary annular position (p=0.003).
Conclusions: Excellent survival follows biventricular repair of pulmonary atresia with intact ventricular septum if RVOTO relief is performed in conjunction with initial palliation. Although most patients eventually require reoperation, avoidance of synthetic transannular patches and monocusp pericardial pulmonary valves may reduce the incidence of wound infection and delay need for pulmonary valve replacement.