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| Year : 2020
| Volume
: 13 | Issue : 4 | Page
: 373-374 |
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| Congenital absence of pericardium |
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Rahul Nikam1, Jordan Rapp2, Ashrith Kandula1, Snigdha Puram1, David Saul1
1 Department of Medical Imaging, A. I. DuPont Hospital for Children, Wilmington, DE, USA
2 Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
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email
| Date of Submission | 08-Jun-2020 |
| Date of Acceptance | 22-Jul-2020 |
| Date of Web Publication | 17-Sep-2020 |
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 Abstract | | |
A 16-year-old girl presented to the emergency department with orthopnea and exercise intolerance for 1 week. Electrocardiogram at that time showed rightward deviation of the cardiac axis, with nonspecific T-wave inversion in the lateral precordial leads and flattening in the inferior leads. She was discharged from the emergency department with cardiology follow-up. Subsequent echocardiogram demonstrated that “the heart is in a funny position in the chest,” and computed tomography of the chest was requested to evaluate for a mediastinal mass causing leftward displacement of the heart.
Keywords: Absent pericardium, congenital, levoposition
How to cite this article:
Nikam R, Rapp J, Kandula A, Puram S, Saul D. Congenital absence of pericardium. Ann Pediatr Card 2020;13:373-4 |
| Differential Diagnosis | |  |
Differential diagnosis includes acute coronary syndromes, cardiac aneurysms, tumors of the lung or heart, mitral valve disease, atrial septal defects, pulmonic stenosis, idiopathic dilation of pulmonary artery (PA), and hilar lymphadenopathy.
| Discussion | | |
The normal pericardium is an avascular sac consisting of two layers: an outer fibrous layer and an inner serosal layer. Serous fluid normally occupies the space between the two layers.
Congenital absence of pericardium (CAP) is a rare condition that results from a failure of the pleuropericardial membranes to fuse completely on one or both sides. CAP has an incidence of <1 in 10,000.[1] Left-sided defects are the most common with a prevalence of 70% of all pericardial defects.[2] Nearly 30%–50% of patients with CAP have other congenital abnormalities, including atrial septal defect, patent ductus arteriosus, and tetralogy of Fallot.[3]
CAP is generally benign but can be confused with other pathologic conditions on imaging. The characteristic findings on chest imaging include a “snoopy dog” appearance of the cardiac silhouette and a teardrop appearance with a bulbous ventricle on chest radiograph (CXR). CXR commonly shows marked levoposition of the cardiac silhouette, loss of the right heart border, prominent PA, and lung tissue between the diaphragm and inferior border of the heart.[4] Scout image from computed tomography (CT) demonstrates loss of right heart border with levoposition of heart [Figure 1]. TTE features of CAP include posterior orientation of the apex, cardioptosis in the parasternal long-axis view[5] [Figure 2]. CT imaging demonstrates interposition of lung tissue between the heart and diaphragm and between the aorta and the main PA [Figure 3]a, [Figure 3]b, [Figure 3]c. | Figure 1: Scout image from computed tomography chest demonstrates obscuration of right heart border by the spine due to levoposition of the heart
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 | Figure 2: Parasternal long-axis transthoracic echocardiogram image showing posteriorly directed apex and unusual imaging windows
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 | Figure 3: (a-c) Two axial and one coronal images from contrast-enhanced chest computed tomography demonstrate interposition of lung tissue in areas of absent pericardium between the aorta and pulmonary artery (straight arrows) and marked levoposition of the cardiac silhouette (curved arrows)
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Patients with partial pericardial defects can be symptomatic, with chest pain noted in one-third of the patients.[6] They are also at risk of complications resulting from strangulation of any herniating cardiac structures. On the contrary, complete absence of pericardium does not commonly cause complications or symptoms.
Although CAP is a rare congenital abnormality, most cases are found incidentally, and it is important for clinicians to recognize the constellation of abnormal clinical and imaging findings for accurate diagnosis.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Yamano T, Sawada T, Sakamoto K, Nakamura T, Azuma A, Nakagawa M. Magnetic resonance imaging differentiated partial from complete absence of the left pericardium in a case of leftward displacement of the heart. Circ J 2004;68:385-8.  |
| 2. | Nasser WK. Congenital diseases of the pericardium. Cardiovasc Clin J 1976;7:271-86. |
| 3. | Centola M, Longo M, De Marco F, Cremonesi G, Marconi M, Danzi GB, Does echocardiography play a role in the clinical diagnosis of congenital absence of pericardium? A case presentation and systemic review. J Cardiovasc Med 2009;10:687-92. |
| 4. | Ellis K, Leeds NE, Himmelstein A. Congenital deficiencies in the parietal pericardium: A review with 2 new cases including successful diagnosis by plain roentgenography. Am J Roentgenol Radium Ther Nucl Med 1959;82:125-37. |
| 5. | Lopez D, Asher CR. Congenital absence of the pericardium. Prog Cardiovasc Dis 2017;59:398-406. |
| 6. | Glover LB, Barcia A, Reeves TJ. Congenital absence of the pericardium. A review of the literature with demonstration of a previously unreported fluoroscopic finding. Am J Roentgenol Radium Ther Nucl Med 1969;106:542-9. |
Correspondence Address:
Dr. Rahul Nikam
Department of Medical Imaging, A. I. DuPont Hospital for Children, 1600 Rockland Rd., Wilmington, DE 19803
USA
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/apc.APC_137_20
[Figure 1], [Figure 2], [Figure 3] |
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