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Age: 0 days (term)

Sex: Male

Indication: Cyanosis

Save ("V")

Case #11


Findings

  • Enlarged cardiothymic silhouette
  • Decreased pulmonary vascularity


Diagnosis

  • Ebstein anomaly

Sample Report

Enlarged cardiothymic silhouette, which in the presence of decreased pulmonary vascularity raises concern for a right-sided heart defect. Recommend cardiology evaluation.

No focal airspace disease, pleural effusion, or pneumothorax.

Unremarkable appearance of the abdomen.


Discussion

  • Ebstein anomaly has a strong associated with maternal lithium therapy during pregnancy
  • This defect results from downward displacement of the posterior and septal leaflets of the tricuspid valve into the right ventricle, creating an atrialized ventricle with resultant tricuspid regurgitation
  • While this defect is often diagnosed in infancy, milder cases are occasionally incidentally discovered in adulthood
  • The most consistent imaging finding is right atrial enlargement, which can be massive resulting in a box-shaped heart
  • As with other right-sided heart defects (like Tetralogy of Fallot and tricuspid atresia), Ebstein anomaly results in decreased pulmonary blood flow
  • Refer to the annotated image below for an algorithmic approach to congenital heart disease on radiographs


Images

Algorithmic approach to congenital heart disease on radiographs.

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