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Ebstein’s Anomaly of the Tricuspid Valve: an Overview of Pathology and Management

  • Valvular Heart Disease (TL Kiefer, Section Editor)
  • Published:
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Abstract

Purpose of Review

Ebstein’s anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population.

Recent Findings

Increased understanding of anatomic accessory atrioventricular pathways in EA has resulted in an improvement in ablation techniques and long-term freedom of atrial arrhythmia recurrence.

Summary

Despite an improvement in understanding and recognition of EA, significant disease heterogeneity and complex treatment options continue to challenge providers, with the best outcomes achieved at expert congenital heart disease centers.

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Authors and Affiliations

  1. Division of Cardiology, Department of Medicine, University of Washington, 1959 Pacific Street, Box 356422, Seattle, WA, 98195, USA

    Mathias Possner, Eric V. Krieger & Zachary L. Steinberg

  2. Division of Cardiothoracic Surgery, University of Washington, Seattle, WA, USA

    Francisco J. Gensini

  3. Division of Cardiac Surgery, Seattle Children’s Hospital, Seattle, WA, USA

    David C. Mauchley

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  1. Mathias Possner
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  2. Francisco J. Gensini
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Correspondence to Zachary L. Steinberg.

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Zachary L. Steinberg reports he is a consultant for Medtronic, but this work has no relationship to the submitted manuscript.

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Possner, M., Gensini, F.J., Mauchley, D.C. et al. Ebstein’s Anomaly of the Tricuspid Valve: an Overview of Pathology and Management. Curr Cardiol Rep 22, 157 (2020). https://doi.org/10.1007/s11886-020-01412-z

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