Congenital absence of the pericardium is rare, and although often asymptomatic, can result in mechanical impairment of cardiac function and even death.
In most cases of isolated absence of the pericardium, patients are asymptomatic.
Complications of congenital pericardial defect may include herniation and entrapment of a cardiac chamber, especially the left atrial appendage.
Associated congenital abnormalities include:
Most partial defects are left sided, and as a result the heart usually rotates toward the left.
- 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
- increased cardiophrenic space is increased on the frontal chest radiograph
Normally, the aortopulmonary window is covered by pericardium and contains some fat. In absence of the pericardium, lung tissue can be detected between the aorta and the main segment of the pulmonary artery.
Occasionally, there is bulging of the left atrial appendage through the defect.
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- 1. Pineda V, Andreu J, Cáceres J et-al. Lesions of the cardiophrenic space: findings at cross-sectional imaging. Radiographics. 27 (1): 19-32. doi:10.1148/rg.271065089 - Pubmed citation
- 2. Wang ZJ, Reddy GP, Gotway MB et-al. CT and MR imaging of pericardial disease. Radiographics. 2003;23 Spec No : S167-80. doi:10.1148/rg.23si035504 - Pubmed citation