Adult Ebstein’s Anomaly: 3D Endoscopic Tricuspid Valve Repair

3D endoscopic correction of Ebstein's anomaly in adults

Surgical treatment of Ebstein's anomaly in adults traditionally relies on cone technique reconstruction or classic ventricular plication. However, the integration of minimally invasive techniques into these complex congenital heart diseases remains a technical challenge. This case report documents the management of a 52-year-old patient suffering from severe tricuspid regurgitation (TR), associated with marked dilation and atrialization of the right ventricle (RV).

The objective of this clinical presentation is to detail an innovative, fully endoscopic tricuspid repair surgical technique under 3D visualization. Unlike circumferential cone reconstruction, the approach preferred here is based on a longitudinal plication of the atrialized segment of the right ventricle coupled with an annuloplasty performed directly at the level of the functional tricuspid annulus.

The hypothesis tested by the surgical team is that in the presence of adequate valvular tissue (Carpentier type A), it is possible to restore competent coaptation without detachment or reimplantation of the leaflets. This strategy aims to avoid the risk of tethering and reduced valvular mobility, while leveraging the benefits of a minimally invasive approach via an incision of only 2.5 cm.

Design and surgical protocol

This case report documents the management of a 52-year-old female patient with Ebstein's anomaly and massive tricuspid regurgitation (TR). The technical challenge? Repairing a valve with a basal ring reaching 52 mm via a fully 3D endoscopic approach, thus bypassing conventional sternotomy for a targeted minimally invasive strategy.

• Configuration chirurgicale : L'accès a été réalisé par une mini-thoracotomie droite de 2,5 cm au niveau du 4e espace intercostal, complétée par deux trocarts. La circulation extracorporelle (CEC) a été établie par canulation fémorale périphérique sous arrêt cardioplégique antérograde.
• Ventricular remodeling: The team performed a longitudinal plication of the atrialized area of the right ventricle using double continuous sutures. This maneuver allows for the exclusion of the dilated portion and the restoration of the functional geometry of the ventricle.
• Reconstruction strategy: Unlike the classic cone technique, the leaflets were not detached. The displaced insertion zone was used as a functional ring for the implantation of a 28 mm semi-rigid partial ring, fixed by 8 U-shaped stitches.
• Optimization and valvuloplasty: An "edge-to-edge" suture was applied between the septal, posterior, and anterior leaflets to ensure coaptation. Sealing was validated by intraoperative filling tests.
• Follow-up and imaging: The precision of the procedure was monitored by immediate transoesophageal echocardiography (TOE), then confirmed by 3D CT scan and transthoracic echocardiography to evaluate the disappearance of atrialization and the transvalvular gradient.

Intraoperative results and technical success

The procedure was successfully performed via a fully endoscopic approach through a 2.5 cm right minithoracotomy. Intraoperative anatomical analysis confirmed major dilatation of the tricuspid annulus associated with a significant atrialized portion of the right ventricle, with apical displacement of the septal leaflet and part of the posterior leaflet.

The surgical strategy relied on a longitudinal plication of the atrialized portion of the right ventricle in order to exclude it from the atrial cavity. The annuloplasty was fixed at the level of the functional annulus, corresponding to the displaced insertion site of the leaflets. A complementary valvuloplasty using the edge-to-edge technique between the septal, posterior, and anterior leaflets optimized the coaptation surface and corrected a residual prolapse of the anterior leaflet identified during the saline leak test.

Postoperative evaluation and follow-up imaging

Transthoracic echocardiography performed before discharge showed residual tricuspid regurgitation qualified as minimal (trace), confirming the competence of the repaired valve without significant valvular stenosis. Overall cardiac function was preserved throughout the immediate postoperative phase.

• Ventricular remodeling: Postoperative computed tomography (CT) confirmed the complete disappearance of the atrialized portion of the right ventricle, validating the effectiveness of the ventricular plication.
• Coronary safety: 3D imaging confirmed the integrity of the right coronary artery, showing no signs of compression or iatrogenic injury related to the ring placement.
• Clinical outcome: The patient was able to return home in good general condition, with resolution of the pre-existing chest discomfort symptoms.

Analysis of endoscopic repair in Ebstein's disease

This clinical case illustrates the viability of a fully endoscopic tricuspid valve repair under 3D visualization in a 52-year-old adult patient. The operative strategy, based on a longitudinal plication of the atrialized portion of the right ventricle and an annuloplasty at the functional ring level, allowed for the restoration of effective coaptation. The choice not to detach the leaflets for a cone reconstruction proved strategic here, avoiding the risk of valvular retraction and loss of mobility associated with short and adherent myocardial interfaces.

The use of a 2.5 cm mini-thoracotomy combined with 3D technology offers a precise surgical alternative for anatomies presenting sufficient valvular tissue. Post-operative CT and ultrasound imaging confirmed the resolution of the right ventricular atrialization and competent valvular function, without clinical stenosis induced by the implantation of the 28 mm semi-rigid partial ring. This technique allows for the preservation of leaflet integrity while correcting ventricular geometry.

The limitations of this study lie in its single case report format. Although the clinical outcome was excellent at patient discharge and during follow-up, the durability of this functional reconstruction compared to the reference technique (da Silva cone reconstruction) remains to be demonstrated. This approach requires confirmed expertise in minimally invasive cardiac surgery (MICS) and rigorous patient selection based on the quality of their valvular tissues.

Summary of results

This clinical case reports the success of a totally endoscopic tricuspid repair via a 2.5 cm minithoracotomy in a 52-year-old patient (Ebstein Type A). The technique, combining longitudinal ventricular plication and annuloplasty on the functional annulus, allowed for discharge on day 8 with minimal tricuspid regurgitation and complete resolution of the right ventricular atrialization confirmed by CT scan.

In concrete terms, for the practitioner:

• Patient selection: This endoscopic approach is particularly suitable for Carpentier types A and B with sufficient and mobile valvular tissue.
• Annuloplasty strategy: In cases of short or adherent leaflet-myocardial junctions, prioritize the implantation of the ring (here 28 mm semi-rigid) at the level of the functional annulus rather than the true annulus to prevent tethering and preserve valvular mobility.
• Geometric restoration: Plication of the atrialized ventricle by double-layer continuous suture is essential to exclude the pathological zone and restore effective right ventricular function under 3D visualization.

Technical lexicon

Ebstein's anomaly: Congenital malformation of the tricuspid valve characterized by an apical displacement of the septal and posterior leaflet attachments toward the apex of the right ventricle.

Atrialized right ventricle: Upper segment of the right ventricle located above the displaced insertion of the tricuspid valve, functioning mechanically as an extension of the right atrium.

Functional tricuspid annulus: Abnormal insertion zone of the valve leaflets within the right ventricle, used in this case as an anchoring site for annuloplasty.

Longitudinal plication: Suture technique aimed at folding the wall of the atrialized right ventricle to reduce its volume and restore a more physiological ventricular geometry.

Edge-to-edge valvuloplasty: Repair technique consisting of suturing the free edges of two valve leaflets to reduce regurgitation by improving the coaptation surface.

Minithoracotomy: Reduced surgical approach (here 2.5 cm) performed in an intercostal space, limiting parietal trauma compared to a median sternotomy.

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