Ebstein Anomaly Guidelines

Updated: Jan 17, 2017
  • Author: Kamran Riaz, MD; Chief Editor: Yasmine Subhi Ali, MD, FACC, FACP, MSCI  more...
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Guidelines

Guidelines Summary

The 2008 American College of Cardiology/American Heart Association (ACC/AHA) 2008 Guidelines for the Management of Adults with Congenital Heart Disease provides detailed information regarding the diagnosis, workup, treatment, follow-up, and instruction regarding nonpregnant, pregnant, and activity level in patients with Ebstein anomaly. [16]  Recommendations for the evaluation and management of patients with Ebstein anomaly are summarized below.

Evaluation

It is recommended that all patients with Ebstein anomaly undergo periodic evaluation at a center with expertise in adults with congenital heart disease (CHD). [16]

Class I

  • Electrocardiography (ECG), radiography, and echocardiography-Doppler ultrasonography are recommended diagnostic studies for Ebstein anomaly in adults.

Class IIa

  • Resting and/or exercise pulse oximetry can be useful in the diagnostic evaluation of Ebstein anomaly in adult patients. (Level of evidence: C)
  • An electrophysiologic study (EPS) can be useful in the diagnostic evaluation of Ebstein anomaly in adult patients with a documented or suspected supraventricular arrhythmia (consider subsequent radiofrequency catheter ablation, if clinically feasible). (Level of evidence: C)
  • For the comprehensive evaluation of Ebstein anomaly in adult patients, Doppler transesophageal echocardiography (TEE) (if transthoracic imaging does not provide sufficient anatomic information); Holter monitoring; EPS for history or ECG evidence of accessory pathway(s); and/or coronary angiography (when planning for surgical repair, if coronary artery disease is suspected, and in men 35 years or older, premenopausal women 35 years or older who have coronary risk factors, and postmenopausal women) can be useful. (Level of evidence: B, for all)

Management

Anticoagulation with warfarin is recommended for patients with Ebstein anomaly who have a history of paradoxical embolus or atrial fibrillation. (Class I, level of evidence: C)

Recommendations for catheter interventions for adults with Ebstein anomaly are summarized below. [16]

  • Catheterizations should be performed at centers with expertise in catheterization and management of adult patients with Ebstein anomaly. (Class I, level of evidence: C)

EPS/pacing issues

  • Catheter ablation may provide benefit in the treatment of recurrent supraventricular tachycardia in some patients with Ebstein anomaly. (Class IIa, level of evidence: B)

Surgical interventions (all class I recommendations)

Surgeons with training and expertise in CHD should perform tricuspid valve repair or replacement with concomitant closure of an atrial septal defect (ASD), when present, for patients with Ebstein anomaly with the following indications (level of evidence: B, for all):

  • Symptoms or deteriorating exercise capacity
  • Cyanosis (oxygen saturation <90%)
  • Paradoxical embolism
  • Progressive cardiomegaly on chest x-ray
  • Progressive right ventricular [RV] dilation or reduction of RV systolic function

Surgeons with training and expertise in CHD should perform concomitant arrhythmia surgery in patients with Ebstein anomaly and the following indications (level of evidence: B, for all):

  • Appearance/progression of atrial and/or ventricular arrhythmias not amenable to percutaneous treatment
  • Ventricular preexcitation not successfully treated in the electrophysiology laboratory

Surgical rerepair/replacement of the tricuspid valve is recommended in adults with Ebstein anomaly with the following indications (level of evidence: B, for all):

  • Symptoms, deteriorating exercise capacity, or New York Heart Association (NYHA) functional class III or IV
  • Severe tricuspid regurgitation (TR) after repair with progressive RV dilation, reduction of RV systolic function, or appearance/progression of atrial and/or ventricular arrhythmias
  • Bioprosthetic tricuspid valve dysfunction with significant mixed regurgitation and stenosis
  • Predominant bioprosthetic valve stenosis (mean gradient greater than 12-15 mm Hg)
  • Operation can be considered earlier with lesser degrees of bioprosthetic stenosis with symptoms or decreased exercise tolerance

Other management considerations

Women of reproductive age

  • Women with Ebstein anomaly should undertake prepregnancy counseling with a physician with expertise in adult CHD. (Class I, level of evidence: C)

Endocarditis prophylaxis

  • It is reasonable to administer antibiotic prophylaxis before dental procedures that involve manipulation of gingival tissue or the periapical region of the teeth or perforation of the oral mucosa in cyanotic patients with Ebstein anomaly and in postoperative patients with a prosthetic cardiac valve. (Class IIa, level of evidence: C)

Problems and pitfalls

Although patients with Ebstein anomaly may be referred for percutaneous or surgical ASD closure, the presence of Ebstein anomaly may alter the recommendation for intervention.

Use caution in performing percutaneous ablation of an accessory pathway in patients with Ebstein anomaly and an interatrial communication with right-to-left shunt, because of the risk of paradoxical embolus.

The presence of multiple accessory pathways should raise the suspicion for Ebstein anomaly.

Patients with Ebstein anomaly and marked cardiomegaly may complain of few symptoms despite marked limitation. Exercise testing will demonstrate functional limitation and should be included as part of the regular assessment of these patients. Exercise testing should include monitoring of oxygen saturation, because exercise-induced cyanosis may occur.

Newly diagnosed patients with Ebstein anomaly may have been told they have concomitant pulmonary arterial hypertension (PAH), particularly in the presence of cyanosis and right-sided heart enlargement. This is usually a misdiagnosis, because PAH is very rare among Ebstein patients.

Other tricuspid valve disorders may be misdiagnosed as Ebstein anomaly.