Ventricularization of the atrialized chamber: A concept of Ebsteinʹs anomaly repair

Background We report results of a technique of Ebsteinʹs anomaly repair by creating a predominantly monocuspid valve with simultaneous ventricularization of the atrialized right ventricular (aRV) chamber. Methods Between March 1993 and April 2003, Ebsteinʹs anomaly repair by valvuloplasty with combined ventricularization was performed in 23 patients aged 13.6 (4.1–52.6) years presenting with tricuspid valve regurgitation (TVR) (I°, n = 1; II°, n = 3; III°, n = 13; IV°, n = 6). Valvuloplasty consisted of creating a predominantly monocuspid valve at the level of the anatomical atrioventricular junction resulting in a ventricularization of the atrialized chamber. Postoperatively all survivors were examined regularly with an actual prospective evaluation. Results One early death (4.4%) occurred and was caused by right heart failure. Follow-up was 4.6 (0.5–10.9) years. Important recurrent atrioventricular valve regurgitation caused by rupture of fixation sutures occurred in 3 patients (13%), necessitating reintervention at 3 (0.03–4) months (revalvuloplasty, n = 2; TV replacement, n = 1). One patient presenting with hypoplastic right ventricle with consecutive right heart failure underwent creation of a total cavopulmonary connection at 10 months. At present all patients are doing well. Actual echocardiographic examination revealed significant improvement of right atrioventricular valve regurgitation (p< 0.0001) and favorable restoration of RV geometry and function. Conclusions This technique of Ebsteinʹs anomaly repair with ventricularization of the atrialized chamber provides excellent results regarding right atrioventricular valve function and leads to a favorable restoration of RV geometry and function.