Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: single-stage complete...

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (MAPCAs) is a complex lesion. Since the concept of primary one-stage unifocalization evolved in the 1990s, the results of surgical treatment have improved significantly. From the midline approach, most of MAPCAs are dissected in the posterior mediastinal space between the ascending aorta and the superior vena cava. Extensive dissection maximizes the length of each MAPCA, which makes direct anastomosis of native tissue feasible without use of prosthetic materials. Pulmonary blood supply is established by a systemic-pulmonary shunt. The procedure was performed in 13 patients with 7.7% of hospital mortality. There was 1 late death because of infection in a patient with deletion of chromosome 22q11.2. Nine patients underwent intracardiac repair without mortality. The ratio of right ventricular systolic pressure to the systemic pressure after intracardiac repair did not exceed 0.5, except for 1 patient. Although further follow-up is necessary, midline one-stage unifocalization is considered as the standard procedure for this lesion.