Revisitation of Double-Inlet Left Ventricle or Tricuspid Atresia With Transposed Great Arteries

Abstract

Patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries are predisposed to systemic outflow tract obstruction necessitating systemic outflow relief operations (SORO). Between January 2000 and February 2018, 30 patients with double-inlet left ventricle (n= 20) or tricuspid atresia (n= 10) with transposition of the great arteries underwent single-ventricle palliation. Arch obstruction was observed in 14 patients. Initial palliative procedures comprised pulmonary artery banding with (n= 4) or without (n= 14) arch repair, bilateral pulmonary artery banding with ductal stenting (n= 5), primary Norwood operation (n= 4), and palliative arterial switch operation (n= 1). Cox proportional hazards model was fitted in 15 patients with initial postnatal echocardiography to identify risk factors for decreased time to SORO. One early and one late death occurred during the median follow-up period of 66 months (10-year survival rate, 93.3%). Various types of SORO were required in 20 of 30 patients (66.7%): Damus-Kaye-Stansel procedure (n= 12), primary Norwood-type palliation (n= 4), palliative arterial switch operation (n= 1), and bulboventricular foramen extension (n= 3). Freedom from SORO at 5 years was 34.5% in all patients (N= 30). Cox regression for the subgroup (n= 15) revealed that arch obstruction (hazard ratio, 20.6; 95% confidence interval, 2.9 to 148.2; p= 0.003) and smaller systemic outflow tract area index at end-systolic phase (hazard ratio, 1.5 at 10mm2/m2 decrease; 95% confidence interval, 1.0 to 2.1; p= 0.033) were identified as risk factors for decreased time to SORO. Arch obstruction and a smaller systemic outflow tract area index at end-systolic phase at initial presentation are predictors of subsequent need for SORO in patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries.