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      Congenital Absence of the Right Pericardium: Embryology and Imaging

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          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.

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          Pericardial disease: value of CT and MR imaging.

          The pericardium represents an important focus of morbidity and mortality in patients with cardiovascular disease. Fortunately, in recent years knowledge regarding this enigmatic part of the heart and the diagnosis of related diseases has substantially advanced. To a large extent, this can be attributed to the availability of several noninvasive cardiac imaging modalities. Transthoracic echocardiography, which combines structural and physiologic assessment, is the first-line technique for examination of patients suspected of having or known to have pericardial disease; however, cardiac computed tomography (CT) and magnetic resonance (MR) imaging are becoming increasingly popular for the study of this part of the heart. Modern multidetector CT scanners merge acquisition speed and high spatial and contrast resolution, with volumetric scanning to provide excellent anatomic detail of the pericardium. Multidetector CT is by far the modality of choice for depiction of pericardial calcifications. MR imaging is probably the best imaging modality for the acquisition of a comprehensive view of the pericardial abnormalities. MR imaging combines cardiac and pericardial anatomic assessment with tissue characterization and appraisal of the effects of pericardial abnormalities on cardiac performance. This review aims to elucidate the role of the pericardium and its interaction with the remainder of the heart in normal and pathologic conditions. It focuses on the rapidly evolving insights regarding pericardial disease provided by modern imaging modalities, not infrequently necessitating reconsideration of evidence that has thus far been taken for granted. http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.13121059/-/DC1. ©RSNA, 2013.
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            Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management.

            Congenital absence of the pericardium (CAP) is a rare clinical entity. We identified from the two hospital databases all patients with isolated CAP, reviewed their data, and invited them for prospective clinical evaluation with electrocardiography, chest x-ray findings (CXR), echocardiography, and magnetic resonance imaging (MRI). Ten patients (3 males, 7 females) presented at a median age of 21 years (range, 2-53 years) with paroxysmal stabbing chest pain, largely nonexertional (9), and heart murmur with an abnormal CXR (1). Three patients had partial and 7 had complete CAP (all 7 had marked lateral displacement of the cardiac apex). CXR combined with MRI were key to establishing the diagnosis; a "tongue" of lung tissue interposing between the main pulmonary artery and aorta was the most consistent diagnostic feature. Four patients underwent pericardioplasty, 3 for debilitating symptoms and 1 for left atrial appendage herniation, followed by improvement or resolution of symptoms. At a mean of 10.5 years from presentation all patients were alive. No complications were seen in the nonsurgical group. Isolated CAP has a common presentation pattern with periodic stabbing chest pain mimicking coronary artery disease. CXR and MRI are required for definitive diagnosis. Symptomatic patients with the complete form may benefit from pericardioplasty.
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              Congenital absence of the pericardium: case presentation and review of literature.

              Congenital absence of the pericardium is an uncommon finding that may or may not be symptomatic. Asymptomatic patients are discovered incidentally during cardiac surgery for an unrelated condition or postmortem. However, symptomatic patients may experience non-exertional paroxysmal stabbing chest pain. It may occur with other cardiac or extracardiac abnormalities and a variety of imaging modalities may identify the condition. Complete cases are more rare than partial effects. However, complications are more common with partial absence due to strangulation of the heart into the defect thus requiring surgical intervention.
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                Author and article information

                Journal
                J Clin Imaging Sci
                J Clin Imaging Sci
                JCIS
                Journal of Clinical Imaging Science
                Medknow Publications & Media Pvt Ltd (India )
                2156-7514
                2156-5597
                2015
                27 February 2015
                : 5
                : 12
                Affiliations
                [1]Department of Radiology, Mayo Clinic, Rochester, Minnesota, United States
                [1 ]Department of Radiology, New York University Langone Medical Center, New York, United States
                Author notes
                Address for correspondence: Dr. Chi Wan Koo, Department of Radiology, Mayo Clinic, 200, 1 st Street Southwest, Rochester - 55905, Minnesota, United States. E-mail: koo.chiwan@ 123456mayo.edu
                Article
                JCIS-5-12
                10.4103/2156-7514.152338
                4374196
                a4102caf-8584-463f-982a-c89ec5024bcf
                Copyright: © 2015 Koo CW.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 04 January 2015
                : 07 February 2015
                Categories
                Pictorial Essay

                Radiology & Imaging
                absence,congenital,embryology,imaging,pericardium,right pericardium
                Radiology & Imaging
                absence, congenital, embryology, imaging, pericardium, right pericardium

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