Ebstein’s Anomaly Annuloplasty

Ebstein's anomaly is a rare cardiac condition classified into four grades based on the extent of atrialization of the right ventricle and the movement of the tricuspid leaflets. This condition is often associated with other anomalies, such as atrial septal defects and Wolff-Parkinson-White syndrome. Surgical intervention is indicated for patients experiencing symptomatic limitations, progressive cyanosis, or arrhythmias. This video highlights the case of an adolescent female undergoing repair of an Ebstein's valve using an annuloplasty ring and septal leaflet resuspension.

This is a case presentation of a 14-year-old female with a history of low oxygen saturations at birth and found to have Ebstein’s anomaly. She was followed for 20 months and noted to have poor growth. Echocardiography showed an apically displaced tricuspid valve with severe regurgitation, a central coaptation defect, a severely dilated right atrium and ventricle, and a compressed left ventricle. Cardiac MRI imaging showed a tethered septal leaflet to the septal wall with restricted motion, the posterior leaflet tethered to the inferior right ventricular wall, and a freely moving anterior leaflet with incomplete coaptation defect consistent with a Type B anomaly. Due to symptomatic limitations of cardiac intolerance, surgical repair was deemed appropriate.

Under general anesthesia, cardiopulmonary bypass was initiated in the standard fashion, maintaining normothermia. After the heart was arrested with Del Nido cardioplegia, the right atrium was then entered with stay sutures placed for better visualization of the tricuspid valve. The valve was inspected using saline to float the valve and was found to be completely incompetent. Pre-operative transesophageal measurements of the valve were 5.2 cm. Further inspection revealed the anterior and septal leaflets were fully delaminated, meaning that the tip of the leaflets were not folded and attached back towards the valve’s hinge point, in anatomically normal fashion. This is indicative of a mild, Type B Ebstein’s anomaly. The septal leaflet was subjectively small. The posterior leaflet sat low in the ventricle, below the annular plane, and was only partially delaminated. Two pledgeted sutures were placed in the commissure of the anterior leaflet and the posterior leaflet to bring the valve tissue together by downsizing the annulus. Nonpledgeted 4-0 Prolene sutures were placed sequentially around the annulus, to which a 32 mm partial ring was implanted. The valve was again tested with saline and found to be more competent but with significant regurgitation. For this reason, the atriotomy was extended between the anterior and posterior leaflets. The atrialized ventricular myocardium was plicated backwards from the most distal extent towards the anatomic annular plane. The valve was re-tested and found to be completely competent. The right atrium was closed, the heart was de-aired, and cross-clamps were removed. Protamine was given, and after adequate hemostasis, the chest was closed in layers with sternal wires for the sternum, Vicryl for the fat and fascia, and Monocryl and Dermabond for the skin.  

Post-Op TEE demonstrated trivial tricuspid valve residual regurgitation, no tricuspid stenosis, a moderately dilated right ventricle with normal systolic function, and a normal left ventricle cavity size and systolic function. The patient was extubated immediately after surgery and then sent to the CVICU for continual recovery. On post-op day 2, chest tubes were removed, and she was discontinued from AV pacing with a return of normal sinus rhythm. Then, after just under 4 days in the CVICU post-op, she was discharged from the unit to head home.

This video provides an overview of Ebstein's Anomaly, outlining its classification and the indications for surgical intervention. Ebstein’s anomaly is categorized into four grades (Carpentier et al 1988): Type A: The volume of the true right ventricle is adequate. Type B: There is a large atrialized portion of the right ventricle, although the anterior leaflet moves freely. Type C: The anterior leaflet is severely restrictive, potentially causing right ventricular outflow tract obstruction (RVOTO). Type D: There is almost complete atrialization of the ventricle. Common anomalies associated with Ebstein's anomaly include atrial septal defects and pre-excitation pathways, such as Wolff-Parkinson-White syndrome (Carpentier et al 1988). Patients should be referred to an electrophysiologist prior to surgery for oblation of any pre-excitation. Surgical intervention is indicated for patients experiencing significant symptoms, progressive cyanosis, or arrhythmias. This video details the repair of an adolescent Ebsteinoid valve using an annuloplasty ring with septal leaflet resuspension.

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There are no acknowledgements.

Carpentier A, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1988;96(1):92-101. doi:10.1016/s0022-5223(19)35302-4. Jonas RA. Comprehensive Surgical Management of Congenital Heart Disease. Taylor and Francis; 2014. Mayo Clinic. Cardiovascular diseases and cardiac surgery. Mayo Foundation for Medical Education and Research. Available at: www.mayoclinic.org/medical-professionals/cardiovascular-diseases/news/cone-reconstruction-for-ebstein-anomaly/mac-20466360.