Ebstein's Anomaly: When Beating-Heart Thoracoscopy Becomes a Game-Changer

Reoperation for Ebstein's anomaly: the challenge of late recurrence

Ebstein's anomaly, a rare congenital malformation of the tricuspid valve, frequently progresses to severe regurgitation and right heart failure. While initial surgical repair often provides prolonged clinical stability, practitioners are regularly faced with late recurrences. This clinical case details the management of a 50-year-old patient presenting with global heart failure (NYHA III, congestive hepatopathy) thirty years after an initial De Vega annuloplasty. The challenge here lies in the complexity of a reoperation in a patient with massively dilated right cavities.

Objectives and hypothesis of the minimally invasive approach

The objective of this report is to demonstrate the technical feasibility of a tricuspid valve re-repair using a totally thoracoscopic approach on a beating heart, under femoro-femoral cardiopulmonary bypass. The study evaluates the efficacy of a "cone" type reconstruction (leaflet mobilization and augmentation with a bovine pericardial patch combined with a 30 mm ring) as an alternative to conventional redo sternotomy. The hypothesis tested is that this minimally invasive approach achieves significant clinical improvement and a reduction in cavity dimensions while limiting surgical aggressiveness, despite the partial loss of echocardiographic benefit observed during long-term follow-up (20 months).

Clinical methodology: Tricuspid re-intervention via beating-heart thoracoscopy

This case report details the surgical strategy adopted for a 50-year-old female patient presenting with recurrent massive tricuspid regurgitation, thirty-one years after a primary De Vega annuloplasty for Ebstein's disease. The patient presented with signs of right-sided heart failure (NYHA III, congestive hepatopathy).

The surgical protocol was based on the following technical parameters:

• Accès et support : Approche totalement thoracoscopique par le côté droit, sous circulation extracorporelle (CEC) fémoro-fémorale. La procédure a été réalisée sur cœur battant.
• Repair technique: Mobilization of the anterior and posterior leaflets, followed by augmentation with a bovine pericardium patch to achieve a "cone" leaflet configuration.
• Annular stabilization: Implantation of a 30 mm prosthetic annuloplasty ring.

The evaluation of results was based on:

• Intraoperative imaging: Transesophageal echocardiography (TEE) to confirm the absence of immediate regurgitation.
• Postoperative follow-up: Clinical evaluations, chest X-rays and transthoracic echocardiographies at 3 months and 20 months. Key measurements included the transverse diameters of the right atrium (64 mm) and the right ventricle (44 mm) at long term.

Surgical Results and Postoperative Evolution

The tricuspid valve re-repair procedure, performed via a total thoracoscopic approach on a beating heart, allowed for immediate correction of the pathology. Intraoperative transesophageal echocardiography confirmed residual tricuspid regurgitation qualified as "trivial" after the implantation of the 30 mm annuloplasty ring and the cone reconstruction using a bovine pericardial patch.

The immediate postoperative course was uneventful, allowing discharge from the facility on the tenth postoperative day (D10). Medium- and long-term follow-up shows a contrasting evolution of valvular function despite persistent cavitary remodeling.

Clinical Observations and Imaging

The 3-month evaluation highlighted a significant improvement in the patient's functional capacity, correlated with a reduction in the dimensions of the right cavities on echocardiography. However, the 20-month data suggest a progressive degradation of valvular sealing:

• Morphological stability: Although cardiomegaly persists on the chest X-ray, it remains less severe than preoperatively. The transverse diameters of the right atrium (64 mm) and the right ventricle (44 mm) confirm the partial maintenance of reverse remodeling.
• Durability of the repair: A partial loss of the initial echocardiographic benefit was noted, with regurgitation progressing from mild (at 3 months) to moderate (at 20 months).
• Clinical signs: Despite this moderate recurrence of the leak, the patient did not present a recurrence of the initial congestive symptoms (ascites, bilateral edema) reported upon admission.

Case analysis and clinical relevance

This clinical case demonstrates the technical feasibility of a re-intervention for Ebstein's anomaly via a totally thoracoscopic approach on a beating heart. In this 50-year-old patient, who had already undergone surgery 31 years earlier via De Vega annuloplasty, this strategy made it possible to bypass the risks inherent in a redo sternotomy in a context of marked right heart failure (NYHA III) and congestive hepatopathy. The use of a bovine pericardial patch for the "cone" reconstruction associated with a 30 mm annuloplasty ring allowed for a rapid recovery, with discharge on the tenth postoperative day.

Limits and durability of re-intervention

The main limitation of this study lies in the partial recurrence of tricuspid leakage over the medium term. While the immediate result was optimal (trivial regurgitation), the 20-month follow-up reveals persistent moderate regurgitation. Although the dimensions of the right cavities remain reduced (RA transverse diameter at 64 mm and RV at 44 mm) compared to preoperative values, this partial loss of the initial benefit highlights the complexity of durably stabilizing a valve that has already undergone surgical remodeling. Unlike conventional sternotomy approaches, the thoracoscopic route offers a less invasive alternative for fragile patients, but the durability of the repair in complex "redo" situations remains a challenge.

Implications for practice

This experience shows that the minimally invasive approach is a serious option for tricuspid valve reoperations, even in rare congenital pathologies. However, the progression from trivial to moderate regurgitation in less than two years requires a cautious interpretation of the longevity of this re-repair technique.

In concrete terms, for the practitioner:

• Alternative to sternotomy: Consider the total thoracoscopic approach on a beating heart for complex tricuspid re-interventions to minimize operative risks in patients who have already undergone multiple surgeries.
• Reconstruction technique: Bovine pericardial patch augmentation remains a viable option for restoring a sufficient coaptation surface, even on previously remodeled valves.
• Vigilance on durability: Do not relax echocardiographic follow-up after initial success; the partial loss of benefit on regurgitation at 20 months requires rigorous long-term monitoring.

Technical lexicon of the study

Ebstein's anomaly: A rare congenital malformation of the tricuspid valve characterized by a deformation of the leaflets, frequently leading to severe tricuspid regurgitation and right-sided heart failure.

De Vega annuloplasty: Surgical technique for valve repair by ring plication, performed on this patient during her initial procedure at the age of 19.

Beating-heart repair: Surgical approach performed without cardioplegic arrest of the heart muscle, used here via thoracoscopy to reduce the risks associated with a re-intervention (redo).

Cone-like configuration: Reconstruction technique consisting of mobilizing and augmenting the anterior and posterior leaflets (via a pericardial patch) to recreate a functional valvular geometry promoting coaptation.

Circulation extracorporelle (CEC) fémoro-fémorale : Méthode de canulation périphérique des vaisseaux fémoraux permettant d'établir le support circulatoire nécessaire à l'approche thoracoscopique totalement endoscopique.

Congestive hepatopathy: Hepatic impairment secondary to systemic venous stasis, observed in this patient due to the severity of preoperative right ventricular failure.

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