What is the best treatment for Ebstein's anomaly?

Treatment of Ebstein's Anomaly

Surgical repair or reoperation is the definitive treatment for Ebstein's anomaly when patients develop heart failure symptoms, objective evidence of declining exercise capacity, or progressive right ventricular systolic dysfunction, with tricuspid valve repair preferred over replacement when anatomically feasible. 1

Treatment Algorithm Based on Disease Severity

Mild Ebstein's Anomaly (Asymptomatic, Normal Heart Size)

• Medical surveillance with regular cardiology follow-up every 12-24 months at centers with congenital heart disease expertise 1
• Monitor for supraventricular arrhythmias, which occur frequently due to accessory pathways present in approximately 25% of patients 1, 2
• Exercise testing should be performed even in subjectively asymptomatic patients, as functional capacity is often objectively reduced 1
• Patients can participate in all sports if heart size is nearly normal and no arrhythmias are present 1

Moderate to Severe Ebstein's Anomaly - Surgical Indications

Class I recommendations for surgical intervention include: 1

• Heart failure symptoms (NYHA class III or IV)
• Objective evidence of worsening exercise capacity on formal testing
• Progressive right ventricular systolic dysfunction documented by echocardiography or CMR
• Cyanosis with oxygen saturation <90%
• Paradoxical embolism
• Progressive cardiomegaly on chest x-ray
• Progressive right ventricular dilation

Class IIa recommendations (reasonable to consider surgery): 1

• Progressive right ventricular enlargement without symptoms
• Systemic desaturation from right-to-left atrial shunt
• Atrial tachyarrhythmias refractory to medical management

Surgical Techniques

Tricuspid Valve Repair (Preferred Approach)

• Cone reconstruction is the current preferred technique, achieving near-anatomic restoration of tricuspid valve anatomy with excellent early and intermediate results 3, 4
• Repair involves creating a monocuspid or bicuspid valve with reduction of the enlarged annulus 5, 6
• Concomitant closure of atrial septal defect or patent foramen ovale when present 1
• Right atrial reduction atrioplasty is often performed 1
• Tricuspid valve repair is preferred over replacement when anatomically feasible, as freedom from reoperation is similar between repair and replacement 6

Tricuspid Valve Replacement

• Indicated when repair is not feasible or repair result is unsatisfactory 1
• Use mechanical or bioprosthetic valve 1
• Reoperation for bioprosthetic dysfunction is recommended when mean gradient >12-15 mm Hg or with symptoms at lesser gradients 1

Adjunctive Procedures

• Bidirectional cavopulmonary (Glenn) anastomosis may be considered when severe right ventricular dilation or dysfunction is present, left ventricular function is preserved, and left atrial/left ventricular end-diastolic pressures are not elevated 1
• This approach is reserved for patients with poor right ventricular function and should not be routine 6

Management of Arrhythmias

Accessory Pathways and Supraventricular Tachycardia

• Catheter ablation is Class I recommended for patients with high-risk pathway conduction or multiple accessory pathways 1
• Electrophysiology study should be performed before surgical repair in all patients with suspected accessory pathways, even without documented arrhythmias 1
• Success rates are lower and recurrence rates higher than in structurally normal hearts due to multiple pathways in nearly 50% of patients 1
• If catheter ablation is unsuccessful, surgical interruption can be performed intraoperatively 1

Atrial Arrhythmias

• Right atrial Maze procedure for atrial flutter history 1
• Biatrial Maze procedure for atrial fibrillation 1

Medical Management

Anticoagulation (Class I Recommendation)

• Warfarin is mandatory for patients with history of paradoxical embolus or atrial fibrillation 1
• The large right atrium predisposes to thrombus formation, and right-to-left shunting at atrial level creates paradoxical embolism risk 1

Heart Failure Management

• Diuretics may reduce peripheral edema but will not improve fatigue and dyspnea related to low left-sided cardiac output 1
• Antiarrhythmic therapy for supraventricular arrhythmias as needed 1

Diagnostic Workup Before Surgery

Essential Imaging

• CMR is useful (Class IIa) to determine anatomy, right ventricular dimensions, and systolic function, particularly when echocardiography is inadequate 1
• TEE is useful (Class IIa) for surgical planning when transthoracic images are inadequate to evaluate tricuspid valve morphology 1
• Coronary angiography before surgery in men ≥35 years, premenopausal women ≥35 years with coronary risk factors, and all postmenopausal women 1

Electrophysiology Evaluation

• Electrophysiology study (Class IIa) is reasonable before surgical intervention even without preexcitation or documented supraventricular tachycardia 1
• Holter monitoring to assess for arrhythmias 1

Special Populations

Neonatal Ebstein's Anomaly

• Represents the most challenging group with high mortality 7
• Treatment options range from observation to valve repair, single ventricle conversion, or cardiac transplantation 5, 7
• Stratification based on clinical status and morphology guides management 5

Reoperation

• Usually requires tricuspid valve replacement or re-replacement 1
• Rerepair of the tricuspid valve is rarely successful 1
• Concomitant Maze procedure for chronic atrial fibrillation/flutter 1

Critical Pitfalls to Avoid

• Do not underestimate tricuspid regurgitation severity due to subtle physical findings and laminar regurgitant flow on echocardiography 1
• Avoid percutaneous ablation with caution in patients with interatrial communication and right-to-left shunt due to paradoxical embolism risk 1
• Do not perform catheterization or surgery at non-expert centers - all procedures should be at centers with expertise in congenital heart disease management 1
• Multiple accessory pathways should raise suspicion for Ebstein's anomaly 1
• Patients with marked cardiomegaly may report few symptoms despite severe limitation - exercise testing is essential 1

Outcomes

• Current era surgical mortality is <3% at experienced centers for children and adults 6
• Late survival and quality of life are excellent for hospital survivors 3
• Atrial tachyarrhythmias are the most common late complication 3
• Poor prognostic factors include NYHA class III/IV symptoms, cardiothoracic ratio >65%, and atrial fibrillation 8