Ebstein Anomaly

  • Etiology: Downwardly displaced tricuspid valve, often with concurrent tricuspid insufficiency, leads to enlarged right atrium and atrialization of right ventricle
  • Imaging: Right to left shunt and right ventricular outflow obstruction leads to cyanosis and decreased pulmonary blood flow
  • Imaging CXR:
    — Massive cardiomegaly with box-shaped (wall to wall) heart
    — Decreased pulmonary blood flow = oligemic lung
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:

Radiology Cases of Ebstein Anomaly

CXR of Ebstein anomaly
CXR AP shows decreased pulmonary vascularity and a huge box-shaped heart.
CXR of Ebstein anomaly
CXR AP shows an extremely enlarged (wall to wall) heart and decreased pulmonary vascularity.