Perioperative care of a child with transposition of the great arteries.

OPINION STATEMENT: Because a minority of patients with D-transposition of the great arteries are diagnosed in utero by ultrasound, most present after delivery with cyanosis. In the absence of apparent lung disease, cyanotic neonates suspected of having a cardiac lesion should be immediately transferred to an intensive care unit at a pediatric tertiary care center for monitoring, resuscitation, and to define the cardiac anatomy and physiology. A prostaglandin E-1 infusion is usually initiated to maintain ductal patency and promote intra-cardiac mixing. In the past, balloon atrial septostomy (BAS) was routinely performed to enlarge the atrial septal defect and improve intra-cardiac mixing while the infants awaited surgery. Recent literature has reported an increase risk of stroke in neonates who undergo BAS, although more recent studies refute this. Our current practice is to perform BAS in neonates who have both echocardiographic evidence of a restrictive atrial septum and hypoxia or instability that is unresponsive to other interventions. The occasional patient who does not respond to initial management may have elevated pulmonary vascular resistance and may stabilize with pulmonary vasodilators, such as inhaled nitric oxide. Rarely, a child does not respond to interventional and pharmacologic resuscitation and requires mechanical support pre-operatively with extracorporeal membrane oxygenation (ECMO). In our experience, ECMO has been a successful bridge to corrective surgery with excellent outcomes. After pre-operative stabilization, arterial switch procedure is typically performed in the first week of life with very favorable early results.