Midterm Results for Collaborative Treatment of Pulmonary Atresia With Intact Ventricular Septum

Background. We report a single-institution experience using a collaborative surgical and catheter-based approach to the initial treatment of pulmonary atresia with intact ventricular septum. Methods. A retrospective review was conducted of all neonates admitted with pulmonary atresia with intact ventricular septum from 1996 to March 2007. Results. We identified 24 patients with a mean age at first intervention of 4.5 days with mean follow-up of 6.05 years (range, 1.9 to 12.7 years). Initial palliation was determined by right ventricular size, morphology, and presence or absence of right ventricular-dependent coronary circulation. Initial catheter-based pulmonary valve perforation and valvuloplasty was performed in 41.7% (10 of 24 patients; group A), and 58.3% (14 of 24 patients) had an initial systemic-to-pulmonary artery shunt (group B). Tricuspid valve size was significantly smaller in group B (median z-score, -0.52 group A versus -2.40 group B; p < 0.001). Placement of a shunt after valvuloplasty in group A was required in 70.0% (7 of 10 patients). There was no mortality in group A, and 70.0% (7 of 10 patients) are in a two-ventricle pathway and 30.0% (3 of 10 patients) are in a 1.5-ventricle pathway. Group B had mortality of 14.3% (2 of 14 patients), both within 5 days of surgery. All group B patients remain in a single-ventricle pathway. Overall survival is 91.7% (22 of 24 patients). Conclusions. An individualized approach to this complex lesion has good results. If the right ventricle can be safely decompressed and appears usable, the need for a shunt after valvuloplasty does not preclude two-ventricle (or 1.5-ventricle) repair. Anatomy mandating a shunt as initial palliation has substantial early mortality.