Repair of Taussig-Bing Anomaly, D-Malposed Great Arteries, and Hypoplastic Aortic Arch

The patient was a 4-day-old, 3.95 kg boy with an antenatal diagnosis of double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly), d-malposed great vessels (side-by-side orientation, aortic valve is rightward and slightly anterior to the pulmonary artery), and severely hypoplastic aortic arch (segment between left common carotid and left subclavian arteries). Essentially, a single coronary arose from the right posterior sinus with an acute marginal artery arising alone from the left anterior sinus.

The patient was born at 39w0d at OSH and was immediately transferred to Cincinnati Children's Hospital Medical Center in Ohio. Due to desaturations, the patient was initiated on PGE-1 at 0.2 mcg/kg/min and was intermittently on continuous positive airway pressure. Surgery indicated for relief of cyanotic congenital heart disease and PGE-1-dependent systemic blood flow. The procedure was as follows: ventricular septal defect baffle closure, ascending aorta and arch reconstruction, and arterial switch operation.