Congenital absence of the pericardium
 Discussion of Congenital absence of the pericardium
- Congenital absence of the pericardium is rare.
- Most pericardial defects are partial and occur on the left side.
- Normally, the aortopulmonary window is covered by pericardium and contains some fat.
- Left-sided absence of the pericardium allows interposition of lung tissue between the aorta and the main segment of the pulmonary artery. Occasionally, there is bulging of the left atrial appendage through the defect.
- The heart usually rotates toward the left.
- Complications of congenital pericardial defect may include herniation and entrapment of a cardiac chamber, especially the left atrial appendage.
- Associated congenital abnormalities:
- Patients who have a pericardial defect without associated congenital abnormalities are often asymptomatic.
 Imaging Findings for Congenital absence of the pericardium
 Plain film
- Apparent elevation of the cardiac apex
- Prominent pulmonary artery segment
- Lucency caused by interposition of the lung between the aorta and main pulmonary artery segment.
- In patients with complete absence of the pericardium, the size of the cardiophrenic space is increased on the frontal chest radiograph.
- Interposition of lung tissue between the aorta and the main segment of the pulmonary artery
 See Also
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 References for Congenital absence of the pericardium
- Víctor Pineda, Jordi Andreu, José Cáceres, Xavier Merino, Diego Varona, and Rosa Domínguez-Oronoz. Lesions of the Cardiophrenic Space: Findings at Cross-sectional Imaging. RadioGraphics 2007 27: 19-32.
- Zhen J. Wang, Gautham P. Reddy, Michael B. Gotway, Benjamin M. Yeh, Steven W. Hetts, and Charles B. Higgins. CT and MR Imaging of Pericardial Disease. RadioGraphics 2003 23: 167S-180S.