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Congenital absence of the right pericardium: embryology and imaging.

Koo CW, Newburg A - J Clin Imaging Sci (2015)

Bottom Line: To date, few short case reports, primarily from the pre-CT and MR era, describe congenital absence of the right pericardium.We present a more comprehensive discussion of the embryologic derangements causing such defects and offer an up-to-date review of characteristic radiologic findings.Recognition of characteristic imaging findings of congenital pericardial absence is crucial in guiding diagnosis and management.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Mayo Clinic, Rochester, Minnesota, United States.

ABSTRACT
Though congenital pericardial absence is often asymptomatic, complications can be life threatening. To date, few short case reports, primarily from the pre-CT and MR era, describe congenital absence of the right pericardium. We present a more comprehensive discussion of the embryologic derangements causing such defects and offer an up-to-date review of characteristic radiologic findings. Recognition of characteristic imaging findings of congenital pericardial absence is crucial in guiding diagnosis and management.

No MeSH data available.


Related in: MedlinePlus

77-year-old man with dyspnea. (a) PA chest radiograph reveals a slightly more horizontal ascending aorta due to partial herniation of the ascending thoracic aorta through a pericardial defect (arrow). (b) Lateral radiograph demonstrates abnormal lucency between the ascending aorta and the right pulmonary artery (arrows) resulting in an unusually dense-appearing and prominent right pulmonary artery (RPA). (c) Transaxial CT imaging with lung window confirms this lucency which represents the lung interposed between the aorta and the pulmonary artery through a partially absent right pericardium (arrow), producing the characteristic “retroaortic air” sign. (d) Coronal reformatted images with soft tissue and lung windows demonstrate air insinuating beneath both the aorta and the pulmonary artery (arrows). The right lung resembles the head and body of an open-mouthed dolphin. (e) Right pericardial absence has been associated with other congenital cardiac defects. Echocardiography demonstrates a secundum type atrial septal defect with left to right shunting (arrow).
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Figure 2: 77-year-old man with dyspnea. (a) PA chest radiograph reveals a slightly more horizontal ascending aorta due to partial herniation of the ascending thoracic aorta through a pericardial defect (arrow). (b) Lateral radiograph demonstrates abnormal lucency between the ascending aorta and the right pulmonary artery (arrows) resulting in an unusually dense-appearing and prominent right pulmonary artery (RPA). (c) Transaxial CT imaging with lung window confirms this lucency which represents the lung interposed between the aorta and the pulmonary artery through a partially absent right pericardium (arrow), producing the characteristic “retroaortic air” sign. (d) Coronal reformatted images with soft tissue and lung windows demonstrate air insinuating beneath both the aorta and the pulmonary artery (arrows). The right lung resembles the head and body of an open-mouthed dolphin. (e) Right pericardial absence has been associated with other congenital cardiac defects. Echocardiography demonstrates a secundum type atrial septal defect with left to right shunting (arrow).

Mentions: Right-sided pericardial defects may manifest radiographically as characteristic cardiac silhouette abnormalities due to herniation of pulmonary and cardiovascular structures through the defect.[26] If the superior aspect of the right pericardium is absent, the interposed lung between the right pulmonary artery and the aorta produces a relative lucency with right pulmonary artery margin prominence [Figures 2a and b]. If the inferior aspect of the right pericardium is absent, the interposed lung may create lucency between the right inferior cardiac border and the right hemidiaphragm.[7] In instances of cardiac tissue herniation through a right pericardial defect, a prominent bulge of the right heart border may be seen [Figure 3a].[2]


Congenital absence of the right pericardium: embryology and imaging.

Koo CW, Newburg A - J Clin Imaging Sci (2015)

77-year-old man with dyspnea. (a) PA chest radiograph reveals a slightly more horizontal ascending aorta due to partial herniation of the ascending thoracic aorta through a pericardial defect (arrow). (b) Lateral radiograph demonstrates abnormal lucency between the ascending aorta and the right pulmonary artery (arrows) resulting in an unusually dense-appearing and prominent right pulmonary artery (RPA). (c) Transaxial CT imaging with lung window confirms this lucency which represents the lung interposed between the aorta and the pulmonary artery through a partially absent right pericardium (arrow), producing the characteristic “retroaortic air” sign. (d) Coronal reformatted images with soft tissue and lung windows demonstrate air insinuating beneath both the aorta and the pulmonary artery (arrows). The right lung resembles the head and body of an open-mouthed dolphin. (e) Right pericardial absence has been associated with other congenital cardiac defects. Echocardiography demonstrates a secundum type atrial septal defect with left to right shunting (arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4374196&req=5

Figure 2: 77-year-old man with dyspnea. (a) PA chest radiograph reveals a slightly more horizontal ascending aorta due to partial herniation of the ascending thoracic aorta through a pericardial defect (arrow). (b) Lateral radiograph demonstrates abnormal lucency between the ascending aorta and the right pulmonary artery (arrows) resulting in an unusually dense-appearing and prominent right pulmonary artery (RPA). (c) Transaxial CT imaging with lung window confirms this lucency which represents the lung interposed between the aorta and the pulmonary artery through a partially absent right pericardium (arrow), producing the characteristic “retroaortic air” sign. (d) Coronal reformatted images with soft tissue and lung windows demonstrate air insinuating beneath both the aorta and the pulmonary artery (arrows). The right lung resembles the head and body of an open-mouthed dolphin. (e) Right pericardial absence has been associated with other congenital cardiac defects. Echocardiography demonstrates a secundum type atrial septal defect with left to right shunting (arrow).
Mentions: Right-sided pericardial defects may manifest radiographically as characteristic cardiac silhouette abnormalities due to herniation of pulmonary and cardiovascular structures through the defect.[26] If the superior aspect of the right pericardium is absent, the interposed lung between the right pulmonary artery and the aorta produces a relative lucency with right pulmonary artery margin prominence [Figures 2a and b]. If the inferior aspect of the right pericardium is absent, the interposed lung may create lucency between the right inferior cardiac border and the right hemidiaphragm.[7] In instances of cardiac tissue herniation through a right pericardial defect, a prominent bulge of the right heart border may be seen [Figure 3a].[2]

Bottom Line: To date, few short case reports, primarily from the pre-CT and MR era, describe congenital absence of the right pericardium.We present a more comprehensive discussion of the embryologic derangements causing such defects and offer an up-to-date review of characteristic radiologic findings.Recognition of characteristic imaging findings of congenital pericardial absence is crucial in guiding diagnosis and management.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Mayo Clinic, Rochester, Minnesota, United States.

ABSTRACT
Though congenital pericardial absence is often asymptomatic, complications can be life threatening. To date, few short case reports, primarily from the pre-CT and MR era, describe congenital absence of the right pericardium. We present a more comprehensive discussion of the embryologic derangements causing such defects and offer an up-to-date review of characteristic radiologic findings. Recognition of characteristic imaging findings of congenital pericardial absence is crucial in guiding diagnosis and management.

No MeSH data available.


Related in: MedlinePlus