Ebstein anomaly

Case contributed by Sandeep Singh Awal
Diagnosis certain

Presentation

Patient presented with history of exertional dyspnea with a murmur on auscultation.

Patient Data

Age: 15 years
Gender: Male
x-ray
  • Marked cardiomegaly with box-shaped appearance.
  • Bilateral lung fields appear unremarkable.
  • Bilateral hemidiaphragms and costophrenic angles appear normal.
  • Bony structures and soft tissues appear unremarkable.

Case Discussion

Echocardiography revaled the following findings:

  • Ebstein anomaly of tricuspid valve showing atrialization of right ventricular cavity with small sized right ventricle.
  • Anterior tricuspid leaflet (ATL) is tethered to interventricular septum
  • Large ostium secundum atrial septal defect with right to left shunt.
  • Grossly dilated right atrium.
  • Low pressure severe tricuspid regurgitation.
  • Good left venticular systolic function.

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