CTICasesCongenital Absence of the Pericardium
Imaging findings
Chest CT and cardiac MRI demonstrate a localized absence of the pericardium along the right ventricular free wall, presenting as a localized outpouching or herniation of the right ventricle into the adjacent mediastinal fat. The superior aspect of the pericardium is present, but there is no normal pericardial sleeve inferiorly.
Key takeaways
Congenital absence of the pericardium is a rare developmental defect that is most commonly left-sided and complete, but can present as a partial defect. Partial defects carry a risk of cardiac herniation and strangulation, presenting as localized bulging of the right or left ventricle through the defect. Multimodality imaging with CT/MRI is diagnostic in demonstrating the focal absence of the pericardial line.
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