Ebstein Anomaly in the Adult Patient: Case Report and Review of the Literature

Abstract

Ebstein's disease is a rare congenital malformation, its prevalence is unknown, anatomically it is characterised by low insertion of one or more leaflets of the tricuspid valve on the right ventricular, reduced size of the right ventricle and tricuspid regurgitation. The diagnosis is made by non-invasive cardiac imaging with transthoracic echocardiography. We Case report a 45-year-old woman followed for pulmonary arterial hypertension and recurrent episodes of heart failure exacerbations. She was admitted to the internal medicine department for investigation of portal thrombosis. After a cardiac re-evaluation, the discovery of severe Ebstein's disease at the surgical stage. The proposed therapeutic decision is a Glenn. The evolution is marked by a rapid clinical worsening of the patient before surgery requiring a transfer in cardiac intensive care unit and then to her death. The aim of this article is to review the manifestations of Ebstein anomaly and to highlight its Mimics. Ebstein's disease is a pathology under-diagnosed or diagnosed late because it is rare and because of the clinical presentation which is similar to other pathologies, hence the need to know how to evoke it in order to make the diagnosis.