Year : 2020  |  Volume : 13  |  Issue : 4  |  Page : 373--374

Congenital absence of pericardium

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

Correspondence Address:
Dr. Rahul Nikam
Department of Medical Imaging, A. I. DuPont Hospital for Children, 1600 Rockland Rd., Wilmington, DE 19803


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.

How to cite this article:
Nikam R, Rapp J, Kandula A, Puram S, Saul D. Congenital absence of pericardium.Ann Pediatr Card 2020;13:373-374

How to cite this URL:
Nikam R, Rapp J, Kandula A, Puram S, Saul D. Congenital absence of pericardium. Ann Pediatr Card [serial online] 2020 [cited 2021 Mar 1 ];13:373-374
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 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.


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}{Figure 2}{Figure 3}

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.

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Conflicts of interest

There are no conflicts of interest.


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