J Cardiovasc Ultrasound.  2014 Mar;22(1):36-39. 10.4250/jcu.2014.22.1.36.

Congenital Absence of the Pericardium

• Kim HJ
• Cho YS
• Cho GY
• Choi SI

• KMID: 2177455
• DOI: http://doi.org/10.4250/jcu.2014.22.1.36

Abstract

Congenital absence of the pericardium is a rare cardiac malformation and is most often asymptomatic. It is usually discovered as an incidental finding. Physical examination, chest radiography, and electrocardiogram are often unremarkable. Echocardiography provides valuable information, and sometimes computed tomography or magnetic resonance imaging is needed for subsequent confirmation.

Keyword

Absence of the pericardium; Complete defect; Partial defect

MeSH Terms

Echocardiography
Electrocardiography
Incidental Findings
Magnetic Resonance Imaging
Pericardium*
Physical Examination
Radiography
Thorax

Figure

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  Fig. 1 A: 12-lead electrocardiogram demonstrating normal sinus rhythm and right deviation of the heart axis. B: Chest radiograph demonstrating leftward position of the heart and the bulging contour of the left heart border. C: Echocardiography apical four chamber view: echocardiography showed a globe-shaped heart and bulbous ventricle. D: Chest CT revealed outpouching of the RV, which contacted the left chest wall (white arrows). E: Chest CT showed interposition of the lung between the aortic arch and pulmonary trunk (yellow arrow). LA: left atrium, LV: left ventricle, RA: right atrium, RV: right ventricle, Ao: aorta.

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  Fig. 2 A: 12-lead electrocardiogram demonstrating sinus rhythm with an incomplete right bundle branch block. B: Chest radiograph demonstrating leftward position of the heart, flattening of the left heart border, and a lucent area between the aorta and pulmonary artery (white arrow). C: Echocardiography apical four chamber view: left ventricular apex showed a swinging motion in diastole and systole. D: Chest CT revealed nonvisualization of the pericardium (yellow arrows) and left-ward displacement of the entire heart with mild right ventricular dilatation.

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  Fig. 3 A: Electrocardiogram demonstrating normal sinus rhythm with left deviation of the heart axis. B: Bulging contour of the left superior cardiac border. C: Apical four chamber view: echocardiography showed a laterally displaced left ventricular apex. D: The cardiac CT revealed absence of the pericardium at the left side of the heart (yellow arrows).

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