What is the management approach for patients with Ebstein anomaly?

Management of Ebstein's Anomaly

The management of Ebstein's anomaly requires a structured approach based on symptom severity, anatomical features, and presence of associated conditions, with surgical intervention recommended for patients with deteriorating exercise capacity, progressive cyanosis, or significant arrhythmias. 1

Diagnostic Evaluation

Initial and Serial Assessment (Class I recommendations)

• ECG: Initial evaluation and every 1-3 years depending on severity 1
• Chest X-ray: Initial evaluation and every 1-3 years 1
• Doppler echocardiography: Initial evaluation and every 1-3 years 1
• Pulse oximetry: At rest and/or during exercise if atrial communication is present 1

Additional Testing

• Exercise testing: Reasonable for initial evaluation and every 1-3 years (Class IIa) 1
  • Important to detect functional limitations that may be underreported by patients 1
  • Must include oxygen saturation monitoring to detect exercise-induced cyanosis 1
• Electrophysiology study: Useful if symptomatic arrhythmias are present (Class IIa) 1
• Holter monitoring: May be considered for asymptomatic patients (Class IIb) 1

Indications for Surgical Intervention

Class I Recommendations (Strongest Indications)

• Deteriorating exercise capacity (NYHA functional class III or IV) 1
• Progressive cyanosis with arterial saturation <80% at rest or with exercise 1

Class IIa Recommendations (Reasonable to Consider)

• NYHA functional class II symptoms if valve appears repairable 1
• Presence of atrial fibrillation 1

Class IIb Recommendations (May Consider)

• Asymptomatic patients with increasing heart size (cardiothoracic ratio >65%) 1
• Asymptomatic patients with stable heart size and arterial saturation <85% when valve appears repairable 1

Surgical Management Options

Tricuspid Valve Procedures

• Tricuspid valve repair: Preferred when feasible 1
  • Reconstruction of the valve is possible, especially with mobile anterior leaflet free of tethering
  • Valvuloplasty with positioning of displaced leaflet to normal level
  • Plication of atrialized portion of right ventricle to reduce size
• Tricuspid valve replacement: When repair is not feasible or unsatisfactory 1
  • Can use mechanical or heterograft bioprosthesis
  • Reoperation usually requires valve replacement as rerepair is rarely successful

Additional Procedures

• Right reduction atrioplasty: Often performed with valve surgery 1
• Glenn anastomosis: Occasionally performed to reduce volume load on right ventricle 1
• Maze procedure: For patients with intermittent or chronic atrial fibrillation/flutter 1, 2
  • Right atrial Maze for atrial flutter
  • Biatrial Maze for atrial fibrillation
• Accessory pathway ablation: Should be mapped and ablated either preoperatively or during surgery 1

Management of Associated Conditions

Arrhythmia Management

• Accessory pathways: Common in Ebstein's anomaly (risk of paroxysmal atrial tachycardia ~25%) 1
  • Multiple accessory pathways present in nearly 50% of patients 2
  • Catheter ablation recommended for recurrent SVT (Class IIa) 2
  • Success rates lower and recurrence rates higher compared to structurally normal hearts 2

Atrial Communication Management

• Closure considerations:
  • Interventional catheterization closure recommended when tricuspid valve is not amenable to repair and patient has exercise intolerance due to hypoxemia 1
  • Should not close atrial communication if significant postoperative TR or RV dysfunction is anticipated 1
  • Caution with percutaneous ablation in patients with right-to-left shunt due to risk of paradoxical embolus 1

Pregnancy Management

• Pre-pregnancy counseling: All women with Ebstein's anomaly should receive counseling with ACHD expert (Class I) 1
• Pregnancy outcomes:
  • Most women can have successful pregnancy with proper care 1
  • Increased risk of low birth weight and fetal loss if significant cyanosis is present
  • Risk of CHD in offspring approximately 6% (without family history) 1

Endocarditis Prophylaxis

• Antibiotic prophylaxis: Reasonable before dental procedures in cyanotic patients and postoperative patients with prosthetic cardiac valves (Class IIa) 1
• Not necessary: In acyanotic, unoperated patients 1

Common Pitfalls and Challenges

• Misdiagnosis of PAH: Pulmonary arterial hypertension is rare in Ebstein patients despite cyanosis and right-sided heart enlargement 1, 2
• Underestimating functional limitation: Patients with marked cardiomegaly may report few symptoms despite significant limitation 1, 2
• Inappropriate ASD closure: Closing atrial septal defect without addressing underlying Ebstein's anomaly can lead to adverse outcomes 2
• Multiple accessory pathways: Failure to recognize can delay diagnosis and appropriate treatment 2
• Other tricuspid valve disorders: May be misdiagnosed as Ebstein's anomaly 1

By following this structured approach to management, patients with Ebstein's anomaly can achieve improved outcomes with appropriate medical and surgical interventions tailored to their specific anatomical and physiological characteristics.