Arterial switch operation with ventricular septal defect repair and aortic arch reconstruction

The arterial switch operation for transposition of the great arteries or double outlet right ventricle with ventricular septal defect (VSD) and aortic arch obstruction is a challenging procedure. One-stage neonatal repair is preferred; however, palliation may be indicated in the newborn who presents with Swiss cheese septum or the patient with extracardiac complications such as necrotizing enterocolitis or subarachnoid hemorrhage. The aortic arch repair is performed with continuous cerebral perfusion and includes patch enlargement of the transverse aortic arch and ascending aorta to control for the important diameter mismatch between the aorta and the pulmonary root. The VSD is no longer closed through the pulmonary valve. Instead, the VSD is approached through the aortic valve after harvesting of the coronary buttons and/or through the tricuspid valve and, if necessary, through an infundibulotomy. The coronary artery transfer is often complex because of the presence of unusual coronary artery patterns and requires precise technique. Subaortic obstruction is often present and requires correction. The right ventricle can be mildly hypoplastic but is not a contraindication to repair unless there is important tricuspid stenosis.