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Ebstein’s anomaly 

Ebstein’s anomaly
Chapter:
Ebstein’s anomaly
Author(s):

Laura Vazquez-Garcia

DOI:
10.1093/med/9780198759447.003.0021
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date: 27 July 2021

Ebstein’s anomaly (EA) is a rare malformation of the tricuspid valve (TV), characterized by apical displacement of the annular attachments of the septal and inferior (mural) leaflets into the right ventricular (RV) cavity, with varying degrees of adherence to the underlying myocardium. The natural history is quite variable and depends on the severity and presence of associated arrhythmias, which plays a significant role with regard to the prognosis. Prenatal diagnosis is associated with higher perinatal mortality, as these usually represent the severe end of the disease spectrum. Different surgical strategies have been employed, although the cone operation, introduced in the past decade, has significantly changed the surgical approach and improved the outcomes of these patients. This chapter presents the case of a newborn with an antenatal diagnosis of EA who presented with severe cyanosis and major arrhythmias at birth requiring intensive care management for the first month of life. After some years of being relatively asymptomatic, he re-presented with recurrent arrhythmias and decreased exercise tolerance, requiring multiple interventions. His clinical course and management are presented, together with a detailed review of this disease from the anatomical aspects to its diagnosis and medico-surgical management.

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