Cone Repair Surgery for Ebstein’s Anomaly in an Adult Patient

Ebstein’s anomaly is a rare congenital heart malformation that accounts for about 0.5 percent of all congenital heart defects. It is characterized by downward displacement of the septal and inferior leaflets of the tricuspid valve, redundant anterior leaflets with a sail-like morphology, dilation of the true right atrioventricular annulus, tricuspid valve regurgitation, and dilation of the right atrium and ventricle. Minor anomalies of the tricuspid valve may not be recognized until adulthood, whereas major anomalies leading to heart failure and cyanosis require surgical intervention earlier in life.

In 1993, Da Silva et al. developed a surgical technique involving cone reconstruction of the tricuspid valve. This operation aims to undo most of the anatomic tricuspid valve defects and to create a cone-like structure with the available leaflet tissue. This technique has become the valvuloplasty of choice, especially beyond the neonatal period. Surgery should be considered in adults when the patient is symptomatic, in the presence of paradoxical embolism, progressive cardiomegaly, progressive right ventricle dilation, or worsening function.



The Patient

This video presents the case of a thirty-three-year-old asymptomatic man who was referred for surgical evaluation for severe tricuspid regurgitation and right ventricular dysfunction. The patient had a history of Ebstein’s anomaly and atrial septal defect diagnosed in childhood, with follow up at another institution.

In 2005, the patient had an ischemic stroke with no sequel and posterior closure of the atrial septal defect with a percutaneous device. A chest radiograph showed increased cardiothoracic ratio secondary to an enlarged right atrium with no other pathological findings. In the transthoracic ultrasound, surgeons found severe right atrium enlargement with impaired right ventricular function. The left ventricular function and diameters were normal.

The ASD closure device was correctly placed but the tricuspid valve showed severe regurgitation with anterior displacement of the septal leaflet with septal adhesions and reduced mobility. The anterior leaflet had normal motion with redundant tissue, and the posterior leaflet was dysplastic and thickened.
According to the echocardiographic findings, the patient met the criteria for a type B Ebstein’s anomaly in the Carpentier’s classification.

Within the spectrum of therapeutic strategies for Ebstein’s anomaly, some patients can be treated with a tricuspid valve surgery exclusively, while more severe patients may need a concomitant right ventricle bidirectional bypass with the Glenn technique, and some might require a total bypass with the Fontan Kreutzer operation when right ventricle dysfunction is the main problem.

In this patient, surgeons believed that tricuspid valve repair with Da Silva’s cone reparation technique was the preferred conduct considering that he had an adequate right ventricular function.

The Surgery

Once surgeons accessed the right atrium, they could see that the ASD closure device was in the right place. Before starting with the tricuspid valve assessment, the team observed the vein of D, marking the atrioventricular node.

When assessing the tricuspid valve, surgeons found a large and mobile anterior leaflet with a small and attached septal leaflet and a small and dysplastic remain of the posterior leaflet. Stay sutures were placed in the free edge of the anterior and septal leaflets.

Next, an incision was made in the anterior leaflet at the 12 o’clock position and was extended in a clockwise fashion, exposing the atrialized ventricle. Surgical delamination of the anterior leaflet toward the posterior leaflet continued by incising fibrous and muscular attachments between the body of the leaflet and the myocardium, preserving the attachments of the leading edge.

Displacement of the septal leaflet in an apical rotational fashion into the right ventricle was then performed. Once the leaflets had been mobilized, the anterior and posterior leaflet were rotated clockwise to meet the edge of the septal leaflet and surgeons sewed them together to form the cone reconstruction.
After the cone was completed, the atrialized ventricle was plicated. In this step, the authors emphasize that it is important to examine the outside of the right ventricle to avoid lesions of the right coronary artery.

Plication of the annulus was then necessary to meet the size of the neotricuspid valve. The leaflet tissue was then anchored to the true annulus with a running suture. Once the cone was complete, a saline test demonstrated competency of the valve. A prosthetic annuloplasty with a number 30 incomplete ring was used.

Intraoperative TEE after cardiopulmonary bypass showed no residual tricuspid regurgitation. The patient was weaned off the ventilator two hours after surgery and discharged on the sixth day after surgery, with an uneventful postoperative course.

References

1. Safi LM, Liberthson RR, Bhatt A. Current Management of Ebstein's Anomaly in the Adult. Curr Treat Options Cardiovasc Med. 2016 Sep;18(9):56. doi: 10.1007/s11936-016-0478-2. PMID: 27439413.
2. Sainathan S, da Fonseca da Silva L, da Silva JP. Ebstein's anomaly: contemporary management strategies. J Thorac Dis. 2020 Mar;12(3):1161-1173. doi: 10.21037/jtd.2020.01.18. PMID: 32274197; PMCID: PMC7139083.
3. Cone Reconstruction of the Tricuspid Valve for Ebstein's Anomaly: Anatomic Repair Dearani, Joseph A. et al. Operative Techniques in Thoracic and Cardiovascular Surgery, Volume 13, Issue 2, 109 - 125
4. Ebstein repair: How I do it. Dearani, Joseph A. JTCVS Techniques, Volume 3, 269 - 276

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