Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: Part I—Anatomy, Physiology, and Palliative Procedures

Abstract

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. Despite vast improvements in the surgical management of this disease, there is still an ongoing controversy regarding the optimal treatment. The purpose of this paper is to summarize the surgical algorithm and experience at Stanford University. We have an experience with more than 300 patients undergoing primary surgical treatment of PA/VSD/MAPCAs. Part I will focus on the anatomy, physiology, and the circumstances in which we utilize palliative procedures for the treatment of PA/VSD/MAPCAs. An aortopulmonary window was utilized in 15% of the patients. Indications for an aortopulmonary window at our center are patients with normally arborizing central pulmonary arteries with dual supply MAPCAs in the presence of cyanosis. Five percent of patients had palliative procedures related to the treatment of a ductus or hemitruncus to one lung and unilateral MAPCAs to the contralateral lung. The combined results achieved for these 2 groups demonstrate an eventual complete repair of 78% and midterm survival of 85%. The surgical treatment of PA/VSD/MAPCAs is made more complicated due to heterogeneity of MAPCA and pulmonary artery anatomy. Using the Stanford algorithm, 20% of patients undergo an initial palliative procedure. The results demonstrate that more than 3-quarters of the patients eventually achieve complete repair.