One and a Half Ventricle Repair With Aortic Valve Repair in an Adult Patient With Ebstein’s Anomaly

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Adult patients with Ebstein’s anomaly remain a challenging subset to manage. In this video demonstrates a one-and-a-half ventricle repair combined with aortic valve repair.  

A 25-year-old female presented with dyspnea on exertion and easy fatigability for the past three months. Echocardiography revealed Ebstein’s anomaly of the tricuspid valve along with a small ostium secundum atrial septal defect. The functional right ventricle was of medium size, and she also had moderate aortic valve regurgitation. 

The patient underwent surgical repair via median sternotomy. After systemic heparinization, cardiopulmonary bypass was initiated using aortic and bicaval cannulation, including high superior vena cava (SVC) cannulation. Moderate hypothermia was maintained, and the aorta was cross-clamped. Antegrade root cardioplegia was administered. 

The right atrium was opened parallel to and approximately 1.5 cm away from the right atrioventricular groove. Right atrial stays were placed. After assessing the anatomy, the anterior tricuspid leaflet (ATL) was detached, starting just beyond the anteroseptal commissure and leaving 2–3 mm of valve tissue near the annulus for future suturing. Similarly, the posterior (PTL) and septal tricuspid leaflets (STL) were detached and delaminated. 

After delaminating all leaflets, the atrialized right ventricle was plicated using 4-0 polypropylene sutures, taking care to avoid coronary injury. Posteroseptal commissuroplasty was performed to reduce the size of the dilated annulus. The ATL was reattached in a double layer using 6-0 polypropylene sutures, with interlocking stitches to prevent a purse string effect. The PTL and STL were reattached in a similar fashion. Fenestrations, if present, were closed using 5-0 or 6-0 polypropylene sutures. During STL reattachment, care was taken to suture just below the vein of D to prevent heart block. 

Following the repair, the tricuspid valve was tested with saline injection, which revealed a central gap with significant tricuspid regurgitation. Therefore, an edge-to-edge repair was performed, approximating the ATL and STL using 5-0 polypropylene sutures. A repeat saline test showed no regurgitation. The tricuspid valve orifice was assessed with a Hegar dilator and found to be adequate. 

For the aortic valve repair, central plication of the noncoronary sinus and subcommissuroplasty between the noncoronary and right coronary commissures were performed. 

Subsequently, a right bidirectional cavopulmonary anastomosis was performed between the SVC and the right pulmonary artery using 6-0 polypropylene sutures, employing an interlocking technique to avoid a purse string effect on the anastomosis. The addition of the cavopulmonary anastmosis provided several benefits, including unloading of the right ventricle and right atrial unloading, which helped mitigate arrhythmias originating from right atrium due to right atrial dilatation. This approach also allowed surgeons to perform more aggressive annuloplasty, as only inferior caval blood was directed to the tricuspid valve (1). After closing the atrial septal defect, the right atrium was closed with 5-0 polypropylene sutures. 

The patient was weaned off cardiopulmonary bypass with stable hemodynamics and an SVC pressure of 11 mm Hg. The postoperative course was uneventful. Thus, one-and-a-half ventricle repair remains a valuable option in carefully selected patients with Ebstein’s anomaly. 

References

1. Malhotra A, Agrawal V, Patel K, Shah M, Sharma K, Sharma P, Siddiqui S, Oswal N, Pandya H. Ebstein's anomaly:" The one and a half ventricle heart". Brazilian journal of cardiovascular surgery. 2018 Jul;33:353-61.

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