What is the best treatment for Ebstein's anomaly?

Best Treatment for Ebstein's Anomaly

Surgical repair or replacement of the tricuspid valve performed by surgeons with expertise in congenital heart disease is the recommended treatment for Ebstein's anomaly when symptoms, deteriorating exercise capacity, cyanosis, or progressive right ventricular dysfunction are present. 1

Indications for Surgical Intervention

Surgical intervention is recommended when one or more of the following are present:

Class I Indications (Strong Recommendation):

• Symptoms or deteriorating exercise capacity 1
• New York Heart Association functional class III or IV 1
• Cyanosis (oxygen saturation less than 90%) 1
• Paradoxical embolism 1
• Progressive cardiomegaly on chest x-ray 1
• Progressive right ventricular (RV) dilation or reduction of RV systolic function 1

Surgical Approach

The primary surgical approach consists of:

1. Tricuspid valve repair when feasible (90% of cases) 1, 2

   • Cone reconstruction has become the preferred technique with promising early and intermediate results 3, 4
   • Modifications may include right ventricular oblique plication, tricuspid valve delamination, surgical valve rotation, and tricuspid ring annuloplasty 3

2. Tricuspid valve replacement when repair is not feasible or unsatisfactory 1

   • Using either mechanical or bioprosthetic valves

3. Additional procedures as needed:

   • Closure of any interatrial communications 1
   • Antiarrhythmia procedures (surgical division of accessory pathways, cryoablation, or Maze procedure) 1
   • Right reduction atrioplasty 1

Special Considerations

For Patients with Severe RV Dysfunction:

• Bidirectional cavopulmonary (Glenn) anastomosis may be considered when:
  • Severe RV dilation or severe RV systolic dysfunction is present
  • LV function is preserved
  • Left atrial pressure and LV end-diastolic pressure are not elevated 1, 5

For Arrhythmia Management:

• Electrophysiological study with catheter ablation is recommended:
  • Before surgical intervention on the tricuspid valve 1
  • For patients with high-risk pathway conduction or multiple accessory pathways 1
  • Success rates for catheter ablation are lower in Ebstein's anomaly (75-89%) compared to structurally normal hearts 1

Medical Management

For patients with mild forms of Ebstein's anomaly not requiring immediate surgery:

• Regular evaluation by a cardiologist with expertise in congenital heart disease 1
• Anticoagulation with warfarin for patients with history of paradoxical embolus or atrial fibrillation 1
• Antiarrhythmic therapy for management of supraventricular arrhythmias 1
• Diuretics may help reduce peripheral edema but will not improve fatigue or dyspnea related to low cardiac output 1

Timing of Surgery

Early surgical intervention is associated with better outcomes:

• Patients who undergo surgery before significant cardiomegaly develops (cardiothoracic ratio >0.6) have better survival and lower reoperation rates 2
• Functional status at the time of surgery is a significant determinant of long-term outcome 2

Follow-up Care

After surgical intervention:

• Lifelong specialized surveillance is required 1
• Monitor for recurrent tricuspid valve dysfunction, prosthetic valve degeneration, arrhythmias, and ventricular dysfunction 1
• Regular follow-up should include echocardiography, ECG, pulse oximetry, and exercise testing 1

Outcomes

• Early mortality: approximately 5-10% in experienced centers 1, 2, 5
• Late survival: 92% at 10 years postoperatively 1
• Freedom from reoperation: 80% at 15 years 1, 61.9% at 20 years 2
• Functional improvement: 90% of patients are in NYHA class I or II after surgery 5

Important Pitfalls to Avoid

• Patients with Ebstein's anomaly may be referred for ASD closure without addressing the underlying tricuspid valve abnormality 1
• Percutaneous ablation of accessory pathways should be performed with caution in patients with right-to-left shunting due to risk of paradoxical embolus 1
• Patients may report minimal symptoms despite significant limitation; exercise testing is essential for objective assessment 1
• Surgical procedures should be performed by congenital heart surgeons at tertiary centers with experience in managing Ebstein's anomaly 1