Ebstein Anomaly: Assessment, Management, and Timing of Intervention

Adult Congenital Heart Disease (A Bhatt and K Niwa, Section Editors)
First Online:
Part of the following topical collections:
  1. Topical Collection on Adult Congenital Heart Disease

Opinion statement

Ebstein anomaly is a developmental abnormality of the tricuspid valve and right ventricle that results in tricuspid regurgitation and right heart enlargement. Because of the variation in clinical severity and associated findings, patients require a detailed, well-tailored evaluation. For these reasons, management of adults with Ebstein anomaly should take place in a center with expertise in adult congenital heart disease. In many patients, the decision regarding if and when to perform surgery remains controversial, largely because of a lack of published data demonstrating improved postoperative symptoms and survival compared to the natural history of the disease. Because standard two-dimensional echocardiography and cardiovascular magnetic resonance imaging planes do not provide the necessary data to preoperatively manage patients, comprehensive echocardiography and cardiovascular magnetic resonance imaging protocols by experts trained in congenital heart disease are essential in the preoperative management of patients with Ebstein anomaly. As patients may be unaware of their exercise limitations, and for prognostic value, serial cardiopulmonary exercise stress testing is very useful in the evaluation of Ebstein anomaly patients. Surgical tricuspid valve repair historically has not been highly successful because of the marked distortion of tricuspid valve leaflets and right ventricular pathology. Over the last several years, reports of newer surgical techniques to repair the valve, with concurrent advances in arrhythmia management of patients hold promise for improved long term outcomes of patients with Ebstein anomaly. However, because Ebstein anomaly is rare and tricuspid valve repair remains technically challenging, the newer valve repair techniques have not yet gained widespread acceptance throughout the adult congenital heart disease community.

Keywords

Ebstein Tricuspid valve Congenital heart disease Cyanosis Adult Pediatric Fetal Arrhythmias Left ventricular noncompaction Patent foramen ovale Atrial septal defect Echocardiography Cardiovascular magnetic resonance imaging Pregnancy 
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Notes

Compliance with Ethics Guidelines

Conflict of Interest

Dr. Puneeta Arya and Dr. Rebecca Beroukhim each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of outstanding importance

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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of Pediatric/Congenital CardiologyMassachusetts General HospitalBostonUSA
  2. 2.Deparment of CardiologyBoston Children’s HospitalBostonUSA

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