What is the Ebstein anomaly?
Ebstein anomaly is congenital displacement of the posterior and septal leaflets of the tricuspid valve toward the right ventricular apex. This results in atrialization of a part of the right ventricle.
Almost always, there is tricuspid insufficiency.
More than 90% of neonates present with cyanosis secondary to an associated right to left atrial level shunt or in the presence of pulmonary valve stenosis or atresia.
Ebstein anomaly comprises 0.5–1.0% of congenital heart disease.
Clinical features of Ebstein anomaly
Severe displacement of the tricuspid valve leaflets presents early in the neonatal and childhood period, while less severe displacement can be tolerated into adulthood.
Neonates with severe Ebstein anomaly and an associated right-to-left atrial level shunt usually present on the first day of life with cyanosis and a tricuspid insufficiency murmur.
Older children or young adults may present with palpitations, a murmur, an abnormal electrocardiogram (ECG), or an abnormal chest radiograph.
Supraventricular tachycardia and Wolff-Parkinson-White syndrome are commonly associated with Ebstein anomaly.
– RAE & RVE
– Elevated LV
– Distorted heart configuration
dilatation of RA with displacement of atrioventricular junction and apical displacement of septal leaflet
Reference)
Charles S. White, Linda B. Haramati, Joseph Jen-Sho Chen, and Jeffrey M. Levsky (2014), Cardiac Imaging, Oxford university press
