Surgical strategies for pulmonary atresia with ventricular septal defect associated with major aortopulmonary collateral arteries.

Treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is challenging and controversial. Basically, the collateral arteries are treated surgically by unifocalization to integrate them and unify the blood flow in the pulmonary circulation. These major collateral arteries are anatomically similar to the bronchial arteries; however, they develop into different vessels because of different environmental exposures. Currently, treatment plans involving surgical intervention in early infancy to address the multiple variations of major aortopulmonary collateral arteries are being established to achieve definitive intracardiac repair. Historically, several surgical approaches have been proposed, including multi-stage unifocalization performed by lateral thoracotomy, rehabilitation of the central pulmonary artery followed by intracardiac repair, and single-stage unifocalization by a midline incision. Recently, single-stage unifocalization performed during infancy has become the preferred method for achieving low right ventricular pressure after closing the ventricular septal defect. Furthermore, for the maintenance of the lowest possible right ventricular pressure after definitive repair, combination therapy or hybrid therapy with catheter and surgical intervention is essential. The surgical treatment strategy for a single ventricle with major aortopulmonary collateral arteries is more difficult than that involving two ventricles.