Ebstein's anomaly

Here, a patient with Ebstein's anomaly. This anomaly has been associated with maternal lithium use during pregnancy, although most cases are sporadic. It is characterized by various degree of displacement of the tricuspid valve towards the apex of the RV, with the posterior and septal leaflets being displaced. This leads to "atrialization" of the RV segment included in the right atrium, with subsequent enlargement of the RA and decreased RV functional cavity.

Ebstein's anomaly is associated with atrial septal defect, Wolff-Parkinson-White (and supra-ventricular tachycardia) and risk of strokes. Often, these patients present with an enlarged cardiac silhouette on chest radiography (“Wall to Wall" heart).

Severe Ebstein's can be associated with the "Circle of death", a flow physiology by which blood flow goes from Right to Left atrium (via the ASD), to the Left ventricle, to the Aorta, to the Pulmonary artery (by the PDA) to the right ventricle (by pulmonary insufficiency), to the right atrium (via the tricuspid regurgitation), to the left atrium by the ASD.

Apical view indicating the enlarged right atrium with the abnormal implantation of the tricuspid valve

Apical view showing the tricuspid regurgitation.

Subcostal view showing the enlarged right atrium

Created by Gabriel Altit - Neonatologist / Créé par Gabriel Altit (néonatalogiste) - © NeoCardioLab - 2020