Congenitally Corrected Transposition of the Great Arteries

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.