A multidisciplinary approach to re-intervention in an Ebstein patient: a case report

Ebstein's anomaly is a rare condition due to incomplete delamination of the tricuspid valve (TV) leaflets with downward displacement of the proximal leaflet attachments. It is associated with a smaller functional right ventricle (RV) and tricuspid regurgitation (TR) that is typically treated with TV replacement or repair. However, future re-intervention poses challenges. We describe a multidisciplinary team approach to re-intervention in a pacing-dependant Ebstein patient with severe bioprosthetic TV regurgitation.

A 49-year-old female patient underwent bioprosthetic TV replacement for severe TR in Ebstein's. Post-operatively, she developed complete atrioventricular (AV) block necessitating the implantation of a permanent pacemaker which included a coronary sinus (CS) lead as the ventricular lead. Five years later, she presented with syncope due to a failing ventricular pacing lead, and a new RV lead was positioned across the TV bioprosthesis due to the lack of CS options. Two years later, she presented with breathlessness and lethargy with severe TR identified on transthoracic echocardiography. She successfully underwent a percutaneous leadless pacemaker implant, extraction of existing pacing system, and implantation of valve-in-valve TV.

Patients with Ebstein's anomaly typically undergo TV repair or replacement. Following surgical intervention, owing to the anatomical location, patients can develop AV block requiring a pacemaker. Pacemaker implantation may involve a CS lead to avoid placing a lead across the new TV in efforts to avoid lead induced TR. Over time, these patients not uncommonly require re-intervention that can be challenging especially in pacing-dependant patients with leads across the TV.