Congenital absence of the pericardium

Congenital absence of the pericardium

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Congenital absence of the pericardium is a rare abnormality.

Normally, the aortopulmonary window is covered by pericardium and contains some fat. Most pericardial defects are partial and occur on the left side. 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.

Associated congenital abnormalities

• Atrial septal defect
• Patent ductus arteriosus
• Mitral stenosis
• Tetralogy of Fallot

Diagnosis

Patients who have a pericardial defect without associated congenital abnormalities are often asymptomatic.

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.

CT

• Interposition of lung tissue between the aorta and the main segment of the pulmonary artery

References

• 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.

See Also

• Pericardial disease
• Cardiac tamponade
• Constrictive pericarditis
• Pericardial effusion
• Pneumopericardium

External Links

• Congenital Absence of the Pericardium
• Goldminer: Congenital absence pericardium

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