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Heart failure in the adult Ebstein patient

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Abstract

Ebstein anomaly comprises approximately 1% of all congenital heart diseases. It occurs when the tricuspid valve fails to properly delaminate from the right ventricle, resulting in a clinical spectrum of abnormal tricuspid valve morphology and right ventricular dysfunction. Due to the anatomy of the tricuspid valve and right ventricle, as well as associated right- and left-sided pathology, patients are at risk for both right and left ventricular failure and the associated symptoms of each. Ebstein patients are also at risk for atrial arrhythmias, due to the atrial enlargement intrinsic to the anatomy, as well as the presence of potential accessory pathways. Arrhythmias are generally poorly tolerated, particularly in the setting of ventricular dysfunction. Cyanosis may also be present in Ebstein patients, due to the common occurrence of atrial communications, which can exacerbate other symptoms of heart failure. Treatment of heart failure can be through pharmacologic and procedural interventions, depending on the underlying cause of heart failure. While early heart failure symptoms may be treated with medical management, most Ebstein patients will require surgery. Various surgical and catheter-based interventions targeting the tricuspid valve and the atrialized right ventricular tissue have been developed to help treat the underlying cause of the heart failure. The optimal timing of transcatheter and surgical intervention in the Ebstein patient to prevent or treat heart failure needs further study.

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Authors and Affiliations

  1. Department of Medicine, Division of Cardiovascular Medicine, Stanford University School of Medicine, 870 Quarry Road – Falk CVRC, Stanford, CA, 94305-5406, USA

    Karen Schultz & Christiane L. Haeffele

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  1. Karen Schultz
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  2. Christiane L. Haeffele
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Correspondence to Karen Schultz.

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Key concepts

1. Ebstein anomaly encompasses a wide range of anatomic variations and severity. There is a bimodal distribution of presentation, with neonates presenting with cyanosis and heart failure, and adults frequently presenting with arrhythmias.

2. Patients can have left or right ventricular failure, and management strategies are unique to the particular cause.

3. Arrhythmia management is of crucial importance in patients with Ebstein anomaly, as patients may not tolerate a non-sinus rhythm well, particularly in the setting of ventricular dysfunction.

4. Atrial communications are common, and require thoughtfulness regarding management, as well as management of other issues in their presence.

5. There are many different strategies for surgical repair, and choice depends on the anatomy of the patient, as well as the expertise of the surgeon.

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Schultz, K., Haeffele, C.L. Heart failure in the adult Ebstein patient. Heart Fail Rev 25, 623–632 (2020). https://doi.org/10.1007/s10741-020-09930-2

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