Neonatal truncus arteriosus repair: surgical techniques and clinical management.

Truncus arteriosus is now ideally repaired in the neonatal period with low morbidity and mortality. Published reports have documented mortality rates in the range of 4% to 5% with mean age at repair as low as 11 days. The physiologic basis for improved outcomes with earlier repair is the avoidance of damaging sequelae of pulmonary overcirculation and heart failure. Data show that baseline mean pulmonary artery pressure is lower in infants undergoing earlier repair. Improved operative outcomes also have been achieved with aggressive truncal valve repair versus replacement in the presence of truncal valve dysfunction, right ventricular outflow tract reconstructive techniques that are patient anatomy-specific, and use of regional perfusion techniques for repair of associated interrupted aortic arch. In addition, a heightened awareness of anomalies of coronary artery ostial location, number, angle of takeoff, and degree of patency can result in avoidance of inadvertent injury to the artery and associated myocardial insult.