The second most common variant of double outlet right ventricle, the anomaly was originally distinguished by a transposed aorta arising entirely from the right ventricle, a pulmonary artery overriding a ventricular septal defect (VSD), side-by-side great vessels, and bilateral subarterial conus with absence of pulmonary-mitral fibrous continuity.1,2 Most surgical series have expanded the definition to include any double outlet right ventricle (with or without pulmonary-mitral continuity) with a subpulmonary VSD, in which left ventricular output flows preferentially to the pulmonary artery. [2][3][4] Despite >30 years of experience with various management strategies, Taussig-Bing anomaly continues to present a considerable surgical challenge. This is largely related to associated anomalies, the most significant of which may be the varying degrees of right ventricular outflow tract and aortic arch obstruction caused by a malaligned ventricular septum. 5 Today, primary arterial switch operation (ASO) with VSD closure is the gold standard for Taussig-Bing anomaly.2,6,7 Significant rates of reintervention, mortality, and neoaortic complications have been demonstrated in studies of this lesion, particularly in patients who had undergone palliative procedures such as pulmonary artery banding before definitive repair. 6,7 Since 1990, single-stage correction with primary ASO has been our institution's practice for all children with Taussig-Bing anomaly. We aimed to describe the long-term outcomes and functional status of patients managed with this surgical approach and to identify factors that predict mortality, reintervention, neoaortic insufficiency (AI), and neoaortic root dilation.
Methods
Patient SelectionAfter obtaining approval from the Institutional Review Board of Columbia University with a waiver of consent, we retrospectively reviewed our hospital and surgical databases for all admissions diagnosed with transposition of the great arteries or double outlet right ventricle. Patients with Taussig-Bing anomaly were identified as those with a transposed aorta and a pulmonary artery arising in Background-Studies of the arterial switch operation for Taussig-Bing anomaly demonstrate significant rates of reintervention and mortality, particularly after initial palliation to delay complete repair. We aimed to describe the longterm outcomes of our 21-year practice of single-stage arterial switch operation for all patients with Taussig-Bing anomaly. Methods and Results-A retrospective study was performed, and 43 patients with Taussig-Bing anomaly were identified between 1990 and 2011. Median age at arterial switch operation was 7 (range, 2-192) days, and median operative weight was 3.2 (1.4-6.2) kg. Aortic arch obstruction was present in 30 patients (70%). Hospital mortality was 7% (n=3). Follow-up was available for 37 hospital survivors at a mean of 8.1 (±6.3) years. Late mortality was 2% (n=1). At follow-up, all patients were in New York Heart Association functional class I. Freedom from transcatheter or surgical reinte...