Ebstein Anomaly in the Adult Patient

Margaret M Fuchs; Heidi M Connolly

doi:10.1016/j.ccl.2020.04.004

Ebstein Anomaly in the Adult Patient

Cardiol Clin. 2020 Aug;38(3):353-363. doi: 10.1016/j.ccl.2020.04.004. Epub 2020 Jun 6.

Authors

Margaret M Fuchs¹, Heidi M Connolly²

Affiliations

¹ Department of Cardiovascular Medicine, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA. Electronic address: fuchs.margaret@mayo.edu.
² Department of Cardiovascular Medicine, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA.

PMID: 32622490
DOI: 10.1016/j.ccl.2020.04.004

Abstract

Ebstein anomaly is a congenital malformation involving primarily the tricuspid valve, with failure of delamination from the underlying myocardium and right ventricular myopathy. Echocardiography is diagnostic in most patients and demonstrates apical displacement of the septal leaflet and variable tethering of leaflet tissue to the right ventricular myocardium. Operative intervention is considered for exertional symptoms, progressive right ventricular enlargement, or right ventricular dysfunction. Tricuspid valve cone repair is the preferred surgical approach. Tricuspid valve replacement and bidirectional cavopulmonary shunt also are considered in patients with advanced disease. Pregnancy generally is well tolerated. Patients with Ebstein anomaly require lifelong follow-up.

Keywords: Accessory pathway; Cone repair; Ebstein anomaly; Pregnancy; Tricuspid valve.

Publication types

Review

MeSH terms

Adult
Cardiac Surgical Procedures / methods*
Ebstein Anomaly* / complications
Ebstein Anomaly* / diagnosis
Ebstein Anomaly* / physiopathology
Ebstein Anomaly* / surgery
Humans
Prognosis
Tricuspid Valve* / abnormalities
Tricuspid Valve* / physiopathology
Tricuspid Valve* / surgery
Ventricular Dysfunction, Right* / diagnosis
Ventricular Dysfunction, Right* / etiology