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

Ann Thorac Surg. 2019 Apr;107(4):1212-1217. doi: 10.1016/j.athoracsur.2018.11.052. Epub 2018 Dec 23.

Abstract

Background: 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).

Methods: 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.

Results: 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 10 mm2/m2 decrease; 95% confidence interval, 1.0 to 2.1; p = 0.033) were identified as risk factors for decreased time to SORO.

Conclusions: 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.

Publication types

  • Comparative Study

MeSH terms

  • Cardiac Surgical Procedures / methods*
  • Cardiac Surgical Procedures / mortality
  • Cohort Studies
  • Double Outlet Right Ventricle / diagnostic imaging
  • Double Outlet Right Ventricle / mortality
  • Double Outlet Right Ventricle / surgery*
  • Echocardiography, Doppler / methods
  • Female
  • Follow-Up Studies
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / surgery
  • Humans
  • Infant
  • Infant, Newborn
  • Kaplan-Meier Estimate
  • Male
  • Palliative Care / methods*
  • Proportional Hazards Models
  • Retrospective Studies
  • Risk Assessment
  • Survival Analysis
  • Transposition of Great Vessels / diagnostic imaging
  • Transposition of Great Vessels / mortality
  • Transposition of Great Vessels / surgery*
  • Treatment Outcome
  • Tricuspid Atresia / diagnostic imaging
  • Tricuspid Atresia / mortality
  • Tricuspid Atresia / surgery*
  • Ventricular Outflow Obstruction / diagnostic imaging
  • Ventricular Outflow Obstruction / mortality
  • Ventricular Outflow Obstruction / surgery*