Congenital Absence of the Right Pericardium with Cardiac Herniation
Imaging findings
CT chest shows a marked rightward contour abnormality of the heart, representing herniation of the right atrium and ventricle through a right-sided defect in the pericardium. Cardiac MRI confirms absence of the right-sided pericardial layer.
Key takeaways
Congenital absence of the pericardium is a rare anomaly. While left-sided defects are more common, right-sided defects can occur and lead to rightward cardiac displacement and herniation, which can cause clinical symptoms from torsion or SVC compression.
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