Successful repair of an ebstein-like malformation of the mitral valve: a case report

Abstract

Background: Ebstein-like malformation of the mitral valve is an exceptionally rare congenital anomaly characterized by incomplete delamination and apical displacement of the posterior mitral leaflet. The resulting deficiency of functional leaflet tissue may cause severe mitral regurgitation and poses a major challenge for valve repair.

Case presentation: A 9-year-old boy presented with fatigue and exertional dyspnea. Echocardiography showed severe mitral regurgitation, marked posterior leaflet hypoplasia, and left atrial enlargement. Intraoperatively, the posterior leaflet was almost completely adherent to the ventricular wall, and the posterior annulus was poorly defined, apically displaced, and dilated. The anterior leaflet and its subvalvular apparatus were preserved. The posterior leaflet and chordal structures were reconstructed using glutaraldehyde-treated autologous pericardium, followed by prosthetic ring annuloplasty. Intraoperative echocardiography demonstrated competent valve closure without mitral stenosis or systolic anterior motion. The postoperative course was uneventful, and mitral valve function remained stable at the one-month follow-up.

Conclusion: Reconstruction of the posterior leaflet and chordal structures using autologous pericardium, combined with ring annuloplasty, may provide a valve-preserving option for pediatric patients with an Ebstein-like malformation of the mitral valve when the anterior leaflet and its corresponding subvalvular apparatus are adequately preserved; although long-term follow-up is required to assess durability