Ebstein's Anomaly

Abstract

Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.

Keywords: Ebstein's anomaly; adult congenital heart disease; arrhythmia; tricuspid valve.

Figure 1.

Tricuspid valve displacement highlights rotation of tricuspid annulus towards the right ventricular outflow tract in addition to downward/apical displacement. ARV: atrialized right ventricle; RA: right atrium; TRV: true right ventricle. Copyright © Mayo Foundation for Medical Education and Research.

Figure 2.

Cardiac magnetic resonance imaging in Ebstein's anomaly. (A) Image of the ventricles in the short-axis plane at the mid-ventricular level. (B) Four-chamber view. (C) Right ventricular inflow-outflow view. The atrialized right ventricle (aRV) is between the atrioventricular junction (dotted line) and the functional tricuspid valve orifice (dashed line). The functional right ventricle (fRV) is the part of the right ventricle between the dashed line and the pulmonary valve. The anatomic right ventricle is a combination of both aRV and fRV. The functional orifice of tricuspid valve is rotated apically and superiorly toward the RV outflow tract. Ao: aorta; LV: left ventricle; PA: pulmonary artery; RA: right atrium. Copyright © Mayo Foundation for Medical Education and Research.

Figure 3.

Key components of cone repair. (A) Standard aortic and bicaval cannulation. Right atriotomy is parallel to the atrioventricular groove. Anatomic examination of tricuspid valve (TV) and atrialized right ventricle (RV). Membranous septum and atrioventricular (AV) node are marked by small vein (vein of D). (B) Delamination of the TV. First incision is made at 12 o'clock in the anterior leaflet a few millimeters away from the true annulus. Surgical delamination is the division of the fibrous and muscular attachments between the body of the leaflet(s) and the free wall of the RV. Delamination must continue to all attachments between the AV groove and the leading edge of the leaflets (close to the RV apex) while keeping the attachments at the leading edge of the leaflet intact. (C) Rotation of leaflet tissue. Following mobilization of all leaflet tissue, the inferior leaflet, or most medial aspect of the anterior leaflet, is rotated clockwise to meet the mobilized septal leaflet. These leaflets are approximated with a monofilament suture (either continuous or interrupted). At completion, the neotricuspid valve orifice should be composed of 360° of leaflet tissue. Prior to reattachment, the atrialized RV is examined to determine need for RV plication. (D) RV plication. Internal triangular plication of portions of the atrialized RV reduces tension on the repair, reduces size of the TV annulus, and eliminates noncontractile RV. Plication is completed with 4-0 or 5-0 monofilament, is started close to the RV apex, and proceeds to the AV groove. Suture line is partial thickness, primarily incorporating the endocardium. Care must be taken to avoid injury to the right coronary artery. (E) Attachment of neo-tricuspid valve to the true annulus. The septal leaflet is reattached to the ventricular septum (just caudal/ventricular side of the true annulus). (F) Repair can be reinforced with either a felt band (in younger children) or an annuloplasty band (older children and adults). CS: coronary sinus; IVC: inferior vena cava; LV: left ventricle; PFO: patent foramen ovale; RA: right atrium; right coronary artery; SVC: superior vena cava; TTA: true tricuspid annulus. Copyright © Mayo Foundation for Medical Education and Research.

Figure 4.

Suture line placement for tricuspid valve repair in Ebstein's anomaly. (A) Coronary sinus (CS) can be left to drain normally into the right atrium (RA) if there is sufficient distance between the coronary sinus and atrioventricular node. (B) If coronary sinus and conduction tissue are close, the suture line can be deviated into the RA to avoid iatrogenic injury, and the coronary sinus then drains into the right ventricle. Copyright © Mayo Foundation for Medical Education and Research.

Figure 5.

Cryoablation for atrial flutter/fibrillation. AVN: atrioventricular node; CS: coronary sinus; FO: foramen ovale; IVC: inferior vena cava; SAN: sinoatrial node; SVC: superior vena cava; TV: tricuspid valve. Copyright © Mayo Foundation for Medical Education and Research.