Cone Repair in Adult Patients with Ebstein Anomaly

Abstract

Ebstein anomaly is a rare congenital heart malformation typically involving the tricuspid valve and the right ventricle that has a wide range of anatomical and pathophysiological presentations. Various surgical repair techniques for Ebstein anomaly have been reported because of its near-infinite anatomical variability. Cone repair for Ebstein anomaly can achieve nearly anatomical reconstruction of the tricuspid valve with promising outcomes. In this article, the surgical techniques for cone repair in adult patients with Ebstein anomaly are described in detail, and clinical experiences and technically challenging cases are presented.

Keywords: Adult patient; Cone repair; Ebstein anomaly.

Fig. 1

Plication of the atrialized ventricle. (A, B) Displaced septal and posterior leaflets; dotted line: the true TV annulus; * displaced septal leaflet; ** displaced posterior leaflet. (C, D) Internal view of plication of the atrialized ventricle; yellow dotted line: interventricular inferior wall bordered by the posterior descending coronary artery; red dotted line: detached line of the posterior leaflet. (E) Posterior annuloplasty; white dotted line: the true TV annulus. (F) External view of the plicated atrialized ventricle; yellow dotted line: the course of the posterior descending coronary artery, black arrowheads: the right coronary artery. TV, tricuspid valve; aRV, atrialized right ventricle; fRV, functional right ventricle; aRVs, the septal area of the aRV; aRVi, the inferior wall of the aRV.

Fig. 2

Surgical delamination. (A) The anterior papillary muscle. (B, C) Displacement of the posterior leaflet and starting surgical delamination; dotted line: the true TV annulus. (D) Detachment of the posterior leaflet; white arrowhead: tethering papillary muscle of the anterior leaflet, black arrowheads: detached line of the posterior leaflet. (E) Mobilized anteroposterior leaflet tissue; white arrow: the anterior papillary muscle acting as the cone apex. (F) Mobilizing the displaced septal leaflets; dotted line: the true TV annulus. TV, tricuspid valve.

Fig. 3

Reattachment of the leaflets. (A) Reattachment of the leaflets clockwise to the true annulus (dotted arrow). (B) Resection of the tethering papillary muscle (arrow). (C) Mobilization of the septal leaflet. (D, E) Saline test using a syringe bulb. (F) Reinforcement of the annulus using a bovine pericardial strip (arrow). CS, coronary sinus.

Fig. 4

Chest CT angiogram of plication of the atrialized ventricle. The top and bottom panels show preoperative and postoperative CT images, respectively. Column (A, E) anterior-posterior view, (B, F) anterior caudal view, (C, G) view from below, and (D, H) posterior caudal view. CT, computed tomography; RA, right atrium; RV, right ventricle; fRV, functional RV; aRV, atrialized RV; LV, left ventricle; LA, left atrium; white and black arrowheads, the right coronary artery running along the atrioventricular groove.

Fig. 5

Chest computed tomography angiogram of plication of the atrialized ventricle in a patient with an abnormal right coronary artery course. (A) Preoperative. (B) Postoperative. Arrowheads: the right coronary artery; red dotted line: the atrioventricular groove; RA, right atrium; RV, right ventricle; fRV, functional RV; aRV, atrialized RV.