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Congenital Complete Absence of the Pericardium

2001; Lippincott Williams & Wilkins; Volume: 103; Issue: 25 Linguagem: Inglês

10.1161/hc2501.092237

1524-4539

Osman Ratib, Joseph K. Perloff, William G. Williams,

Congenital Heart Disease Studies

HomeCirculationVol. 103, No. 25Congenital Complete Absence of the Pericardium Free AccessOtherPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessOtherPDF/EPUBCongenital Complete Absence of the Pericardium Osman Ratib, Joseph K. Perloff and William G. Williams Osman RatibOsman Ratib From the Department of Radiological Sciences (O.M.) and the Ahmanson/UCLA Adult Congenital Heart Disease Center (J.K.P.), University of California, Los Angeles, and the Department of Surgery (W.G.W.), the Toronto Hospital, Toronto, Ontario, Canada. , Joseph K. PerloffJoseph K. Perloff From the Department of Radiological Sciences (O.M.) and the Ahmanson/UCLA Adult Congenital Heart Disease Center (J.K.P.), University of California, Los Angeles, and the Department of Surgery (W.G.W.), the Toronto Hospital, Toronto, Ontario, Canada. and William G. WilliamsWilliam G. Williams From the Department of Radiological Sciences (O.M.) and the Ahmanson/UCLA Adult Congenital Heart Disease Center (J.K.P.), University of California, Los Angeles, and the Department of Surgery (W.G.W.), the Toronto Hospital, Toronto, Ontario, Canada. Originally published26 Jun 2001https://doi.org/10.1161/hc2501.092237Circulation. 2001;103:3154–3155A 38-year-old woman with ill-defined chest pain had an abnormal x-ray 8 years before her only pregnancy. She had been unable to bear down during delivery, but gestation was otherwise uncomplicated, yielding a normal female offspring. She subsequently experienced left-sided chest pain that awakened her from sleep when turning into either a right or left lateral decubitus position. The pain was pulsating, stabbing, heavy, moderate in severity, and sometimes lasted for hours. Thoracic MRI in the supine and left lateral recumbent positions diagnosed congenital complete absence of the pericardium (Figure 1, left). The pain was ascribed to torsion at the thoracic inlet because of striking positional changes (mobility) of the heart (Figure 1, left). Congenital complete absence of the pericardium was confirmed during an operation designed to stabilize the cardiac position (Figure 1, right, and Figure 2). The heart was encapsulated in a Gore-Tex sack. The postoperative magnetic resonance images are shown in Figures 1 and 2. Figure 3 shows hypoplasia of the left lung and left pulmonary artery, which sometimes coexist with congenital complete absence of the pericardium. Download figureDownload PowerPoint Figure 1. Axial views. The preoperative (PreOp) image shows the mobile, unfixed heart. The postoperative (PostOp) image shows adhesion (fixation) of the lateral wall of the right ventricle to the chest wall (arrows) after surgical anchoring. RA indicates right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle; and Ao, aorta.Download figureDownload PowerPoint Figure 2. Dynamic images obtained in a horizontal long axis (4-chamber) view in diastole and systole with the patient positioned on her right side. These images illustrate the absence of significant cardiac motion after surgical fixation of the heart to the chest wall (arrows). Abbreviations as in Figure 1.Download figureDownload PowerPoint Figure 3. Three-dimensional reconstruction of gadolinium-enhanced MR angiography of the heart and great arteries in the anterior (ANT) and left anterior oblique (LAO) views. The left anterior oblique view shows the hypoplastic left pulmonary artery (LPA). RPA indicates right pulmonary artery; Ao, aorta; PA, pulmonary artery; S, superior; and I, inferior.FootnotesCorrespondence to Joseph K. Perloff, MD, Division of Cardiology, Room 47-123, UCLA Center for the Health Sciences, 10833 LeConte Avenue, Los Angeles, CA 90095-1679. E-mail [email protected] eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. Authors of the article cited in the comment will be invited to reply, as appropriate.Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page.Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetailsCited By Marelli A and Aboulhosn J (2023) Congenital abnormalities of the pericardium Perloff's Clinical Recognition of Congenital Heart Disease, 10.1016/B978-0-323-52964-8.00014-4, (48-52), . Komorovsky R, Palamarchuk Y, Lubkovych O, Palamar T and Hladkykh F (2021) Multimodality imaging and clinical significance of isolated congenital absence of the pericardium, Український радіологічний та онкологічний журнал, 10.46879/ukroj.3.2021.116-124, 29:3, (116-124) Perloff J and Marelli A (2012) Congenital Abnormalities of the Pericardium Clinical Recognition of Congenital Heart Disease, 10.1016/B978-1-4377-1618-4.00005-5, (51-55), . Zoroufian A, Shirani S, Eslami B and Sahebjam M Congenital total absence of the pericardium: A case report, Open Medicine, 10.2478/s11536-009-0018-5, 5:3, (315-317) Garnier F, Eicher J, Philip J, Lalande A, Bieber H, Voute M, Brenot R, Brunotte F and Wolf J (2009) Congenital Complete Absence of the Left Pericardium: A Rare Cause of Chest Pain or Pseudo-right Heart Overload, Clinical Cardiology, 10.1002/clc.20607, 33:2, (E52-E57), Online publication date: 1-Feb-2010. Centola M, Longo M, De Marco F, Cremonesi G, Marconi M and Danzi G (2009) Does echocardiography play a role in the clinical diagnosis of congenital absence of pericardium? A case presentation and a systematic review, Journal of Cardiovascular Medicine, 10.2459/JCM.0b013e32832b3d4a, 10:9, (687-692), Online publication date: 1-Sep-2009. PERLOFF J (2009) Survival Patterns Without Cardiac Surgery or Interventional Catheterization: A Narrowing Base Congenital Heart Disease in Adults, 10.1016/B978-1-4160-5894-6.50008-3, (25-67), . St. Peter S, Shah S, Little D, Calkins C, Sharp R and Ostlie D (2005) Bilateral congenital diaphragmatic hernia with absent pleura and pericardium, Birth Defects Research Part A: Clinical and Molecular Teratology, 10.1002/bdra.20173, 73:9, (624-627), Online publication date: 1-Sep-2005. Duerinckx A (2004) Plain film / MR imaging correlation in heart disease, Radiologic Clinics of North America, 10.1016/j.rcl.2004.03.001, 42:3, (515-541), Online publication date: 1-May-2004. June 26, 2001Vol 103, Issue 25 Advertisement Article InformationMetrics Copyright © 2001 by American Heart Associationhttps://doi.org/10.1161/hc2501.092237 Originally publishedJune 26, 2001 PDF download Advertisement