From the Guidelines
Ebstein's anomaly with severe tricuspid regurgitation should be treated with surgical intervention, specifically tricuspid valve repair or replacement, as soon as symptoms or deteriorating exercise capacity occur, to improve morbidity, mortality, and quality of life. The treatment approach involves a combination of medical management and surgical intervention, depending on symptom severity and patient factors. Medical treatment typically includes diuretics, ACE inhibitors, and beta-blockers to control symptoms and prevent complications. However, surgical intervention is the definitive treatment for severe cases, with the goal of reducing regurgitation, improving right ventricular function, and preventing progressive right heart failure.
Surgical Intervention
Surgical repair or replacement of the tricuspid valve is recommended for patients with Ebstein's anomaly and severe tricuspid regurgitation, as it can improve symptoms and functional ability, and prevent or delay worsening symptoms 1. The primary operation generally consists of closure of any interatrial communications, antiarrhythmia procedures, and tricuspid valve surgery. Tricuspid valve repair is preferred when feasible, and tricuspid valve replacement is performed with a mechanical or heterograft bioprosthesis when repair is not feasible or the repair result is not satisfactory.
Indications for Surgery
Surgery is typically recommended when patients develop symptoms like exercise intolerance, heart failure, cyanosis, or paradoxical embolism 1. The timing of surgery is crucial, and it should occur before significant right ventricular dysfunction develops, as this improves long-term outcomes. Additionally, surgery may be considered earlier in patients with severe RV dilation or severe RV systolic dysfunction, preserved LV function, and low left atrial pressure 1.
Additional Procedures
Other procedures that may be performed concomitantly with tricuspid valve surgery include arrhythmia surgery, such as a modified right atrial maze procedure, and closure of atrial level shunt. Bidirectional cavopulmonary anastomosis may be considered in selected patients with severe RV dysfunction and preserved LV function with low left atrial pressure 1. Heart transplantation may be considered when significant LV dysfunction has occurred and important symptoms of heart failure are present 1.
Key Considerations
The management of patients with Ebstein's anomaly should be in tertiary CHD centers or children's hospitals with experienced medical and surgical personnel. Surgeons with training and expertise in CHD should perform operations for patients with Ebstein's anomaly. The use of catheter ablation is recommended for adults with Ebstein anomaly and high-risk pathway conduction or multiple accessory pathways 1.
From the Research
Treatment Options for Ebstein's Anomaly with Severe Tricuspid Regurgitation
- Surgical correction is the primary treatment for Ebstein's anomaly with severe tricuspid regurgitation, which can include tricuspid valve repair or replacement 2, 3, 4.
- Tricuspid valve repair techniques have evolved over time, with various methods reported in the literature, including cone reconstruction, which aims to restore the tricuspid valve anatomy 2.
- The choice of surgical technique depends on the individual patient's anatomy and the severity of the anomaly 3, 5.
- Tricuspid valvuloplasty (TVP) and tricuspid valve replacement (TVR) are two common surgical approaches, with TVP being considered the first-choice treatment due to its lower mortality rate and improved outcomes 6.
Surgical Outcomes and Follow-up
- Studies have reported excellent long-term survival and quality of life for patients with Ebstein's anomaly who undergo surgical correction, with overall survival rates ranging from 91.26% to 97.9% at 5-20 years 6, 5.
- Regular follow-up is essential to monitor for signs and symptoms of heart failure, arrhythmias, and other associated findings, and should be performed in collaboration with an adult congenital heart center of excellence 4.
- Exercise tolerance and physical activity are important aspects of management, with patients encouraged to engage in physical activity as tolerated, although those with severe anomalies may need to avoid competitive sports 4.
Management of Associated Conditions
- Atrial tachyarrhythmias are a common late complication of Ebstein's anomaly, and management of arrhythmias is an essential aspect of treatment 2, 3.
- Patients with Ebstein's anomaly and associated conditions, such as Wolff-Parkinson-White syndrome, may require one-stage surgery to address these issues 6.
- Severe left ventricular outflow tract obstruction (LVOTO) during surgery may require reoperation of the atrialized right ventricle 6.