Congenitally corrected transposition of the great arteries: Ventricular function at the time of systemic atrioventricular valve replacement predicts long-term ventricular function

Objectives: The objective was to evaluate the systemic ventricular ejection fraction (SVEF) at the time of systemic atrioventricular valve (SAVV) replacement as a predictor of SVEF ≥1 year after surgery in patients with congenitally corrected transposition of the great arteries (CCTGA). Background: Progressive SAVV regurgitation causes systemic ventricular failure in CCTGA patients, who are commonly referred late for intervention. Survival after surgery is poor when the pre-operative SVEF is <44%. Methods: We retrospectively reviewed 46 patients (pre-operative SVEF ≥40% in 27 patients and <40% in 19 patients) with 2 good-sized ventricles, a morphologically right systemic ventricle, and SAVV regurgitation requiring surgery. Median follow-up was not different in patients with a pre-operative SVEF ≥40% (8.8 years) or <40% (7.7 years, p = 0.36). Results: Pre-operative SVEF was the only independent predictor of ≥1-year post-operative SVEF (p < 0.0001). The late SVEF was preserved (defined as ≥40%) in 63% of patients who underwent surgery with an SVEF <40% compared with 10.5% of patients who underwent surgery with an SVEF <40%. Pre-operative variables associated with late mortality were an SVEF ≤40%, a subpulmonary ventricular systolic pressure ≥50 mm Hg, atrial fibrillation, and New York Heart Association functional class III to IV. Conclusions: Post-operative systemic ventricular function after SAVV replacement can be predicted from the pre-operative SVEF. For best results, operation should be considered at an earlier stage, before the SVEF falls below 40% and the subpulmonary ventricular systolic pressure rises above 50 mm Hg.