All::Cardiovascular System::Anatomy::Ebstein's anomaly

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What is Ebstein's anomaly?

a congenital heart defect characterised by low insertion of the tricuspid valve resulting in a large atrium and small ventricle

Ebstein's anomaly

What can cause Ebstein's anomaly?

may be caused by exposure to lithium in-utero

Ebstein's anomaly

What other cardiac abnormalities are associated with Ebstein's anomaly?

  • patent foramen ovale (PFO) or atrial septal defect (ASD) is seen in at least 80% of patients, resulting in a shunt between the right and left atria
  • Wolff-Parkinson White syndrome

Ebstein's anomaly

What are the clinical features of Ebstein's anomaly?

  • cyanosis
  • prominent 'a' wave in the distended jugular venous pulse,
  • hepatomegaly
  • tricuspid regurgitation
  • pansystolic murmur, worse on inspiration
  • right bundle branch block → widely split S1 and S2

Ebstein's anomaly