Ventricularization of the Atrialized Chamber: A Concept of Ebstein’s Anomaly Repair

BACKGROUND: We report results of a technique of Ebstein’s 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, Ebstein’s 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 Ebstein’s 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.