Surgical Outcomes of Repairing Ebstein Anomaly: A Single-Center Experience

Background: Ebstein anomaly (EA) is a rare congenital disorder of the tricuspid valve (TV), accounting for about one percent of congenital heart defects. Considering highly variable anatomy, diverse corrective surgical methods are currently utilized. Cone reconstruction provides a near anatomic tricuspid restoration method that has triggered enthusiasm for early repair over recent years. Objectives: In the current study, we aimed to evaluate the clinical and echocardiographic features and outcomes of patients who underwent surgical correction of EA in our referral center. Methods: In this study, we reviewed the medical records of 35 patients with EA who underwent cone repair or TV replacement and had accessible echocardiographic data before and at least one year following the corrective surgery. The patients were evaluated for residual tricuspid regurgitation (TR) severity, right ventricular (RV) size, RV fractional area change (FAC), left ventricular end-systolic diameter (LVESD), left ventricular end-diastolic diameter (LVEDD), and 2D left ventricular ejection fraction (LVEF) before and after the operation. Results: Mean age of the patients was 29.49 ± 9.13 years, and 54.3% of them were female. Twenty-one (60%) patients underwent cone repair, and 14 were subjected to TV replacement. Seven (20%) patients required reoperation; redo TV repair was conducted in three patients (8.6%); two (5.7%) patients underwent biologic TV replacement; mechanical TV replacement was performed in one patient (2.9%) who had degenerative biologic TVs, and one patient (2.9%) was subjected to redo-operation due to mechanical TV malfunction. Positive changes in the means of RV-FAC and 2D-LVEF were statistically significant in all patients irrespective of the type of surgery (P < 0.001), but changes in the means of RV size (P = 0.38) and LVESD (P = 0.302) were not statistically significant. There was a significant increase in functional RV size in both groups of patients who underwent repair (P = 0.063) or replacement (P = 0.02). Conclusions: The present study revealed satisfactory post-op results for cone TV repair in patients with EA, evidenced by reduced TR severity and improved biventricular functional parameters. Assessing the durability of the outcomes of cone TV repair needs longer-term follow-ups.