Pericardial agenesis

Case contributed by Maxime St-Amant

Presentation

Tachyarrhythmia.

Patient Data

Age: 55 years
Gender: Female
x_ray

Levoposition of the heart. Radiolucency between the inferior cardiac border and the left hemidiaphragm. The pulmonary artery is not significantly enlarged. The findings are suggestive of partial left pericardial agenesis. Cardiac CT or MRI is recommended.

ct

Cardiac CT-scan confirms levoposition of the heart and posterior location of the apex. There is a partial, but almost complete pericardial agenesis, with subtle pericardial remain overlying the right atrium. Note is also made of a tongue of lung tissue between the aorta and pulmonary artery (at the level of pulmonary trunk bifurcation).

mri

Cardiac (two chamber cine view) MRI confirms levoposition of the heart and posterior location of the left heart.

Case Discussion

Pericardial agenesis (partial) is a rare cardiac defect. Radiologically, it presents with the following :

  • levoposition of the heart
  • prominent pulmonary artery
  • air interface in the aorto-pulmonary window or between the base of the heart and the diapgragm

CT-scan is better than MRI for this diagnosis, since it has better spatial resolution. The pericardial defect will be seen. A complete pericardial defect is a benign condition, while patients with partial agenesis may have cardiac herniation through the defect. They should be managed surgically.

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