Partial Congenital Absence of the Pericardium
2007; Lippincott Williams & Wilkins; Volume: 116; Issue: 6 Linguagem: Inglês
10.1161/circulationaha.107.701599
ISSN1524-4539
AutoresMichaela Scheuermann‐Freestone, Elizabeth Orchard, Jane M Francis, MARK E.V. PETERSEN, Matthias G. Friedrich, Abbas Rashid, Darryl F. Shore, Saul Myerson, Stefan Neubauer,
Tópico(s)Cardiomyopathy and Myosin Studies
ResumoHomeCirculationVol. 116, No. 6Partial Congenital Absence of the Pericardium Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBPartial Congenital Absence of the Pericardium Michaela Scheuermann-Freestone, MD, DPhil, Elizabeth Orchard, MD, Jane Francis, DCR(R), DNM, Mark Petersen, MD, Matthias Friedrich, MD, Abbas Rashid, MD, Darryl Shore, MD, Saul Myerson, MD and Stefan Neubauer, FRCP FACC Michaela Scheuermann-FreestoneMichaela Scheuermann-Freestone From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. , Elizabeth OrchardElizabeth Orchard From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. , Jane FrancisJane Francis From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. , Mark PetersenMark Petersen From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. , Matthias FriedrichMatthias Friedrich From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. , Abbas RashidAbbas Rashid From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. , Darryl ShoreDarryl Shore From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. , Saul MyersonSaul Myerson From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. and Stefan NeubauerStefan Neubauer From the University of Oxford Centre for Clinical Magnetic Resonance Research (M.S.-F., J.F., S.M., S.N.), John Radcliffe Hospital, Oxford, United Kingdom; Gloucester Royal Hospital (E.O., M.P.), Gloucester, United Kingdom; Stephenson Clinical Magnetic Resonance Centre (M.F.), University of Calgary, Alberta, Canada; and the Department of Cardiac Surgery (A.R., D.S.), Royal Brompton Hospital, London, United Kingdom. Originally published7 Aug 2007https://doi.org/10.1161/CIRCULATIONAHA.107.701599Circulation. 2007;116:e126–e129is corrected byCorrectionA 45-year-old male was admitted to the hospital with recurrent, stabbing, nonexertional chest pain. Chest x-ray showed an increased cardiothoracic ratio (Figure 1A), and ECG demonstrated sinus rhythm with incomplete right bundle-branch block and poor R-wave progression (Figure 1B). Echocardiography showed normal left ventricular dimensions and function; however, the right ventricle appeared grossly dilated with moderate to severe tricuspid regurgitation with no evidence of intracardiac shunts. These findings were confirmed by right heart catheterization, which also showed normal pulmonary and right atrial pressures without a pressure gradient over the pulmonic valve. Catheterization of the left heart showed normal ventricular pressures and function as well as normal coronary arteries. The patient was referred for cardiac magnetic resonance imaging (CMR) with suspected right ventricular cardiomyopathy. CMR showed cardiac displacement into the left hemithorax (Figure 2A). There was severe tricuspid regurgitation with marked dilatation of both the right atrium (6×8 cm) and ventricle (end-diastolic volume, 343 mL). Right ventricular function was mildly reduced (ejection fraction, 46%) without localized wall motion abnormalities. Left ventricular volumes and function were normal; however, there was marked indentation of the mid-lateral wall that resulted in herniation of the apical portion of the left ventricle without associated wall-motion abnormalities (Figure 2B). In addition, there was an extension of lung tissue between the inferior surface of the heart and the diaphragm as a result of the unusually posterior displacement of the left ventricle (Figure 3). Late enhancement images were normal, which made a diagnosis of right ventricular cardiomyopathy less likely. Download figureDownload PowerPointFigure 1. A, chest x-ray shows increased cardiothoracic ratio. B, Twelve-lead ECG shows incomplete right bundle-branch block and poor R-wave progression.Download figureDownload PowerPointFigure 2. A, marked displacement of the heart into the left hemithorax with increase in right atrial and ventricular size. Image acquired with a cardiac-gated HASTE sequence (nonbreathhold; trigger pulse, 2; slice thickness, 7 mm) with a flip angle of 160°; TR, 800 ms; TE, 24 ms; and a matrix of 256×164. B, herniation (arrow) of the apical left ventricular wall. Image acquired with a cardiac gated TrueFISP cine sequence (breathhold; slice thickness, 7 mm) with a flip angle of 60°; TR, 3 ms; TE, 1.51 ms; and a matrix of 256×164. See online Data Supplement for Movie. LV indicates left ventricle; RV, right ventricle.Download figureDownload PowerPointFigure 3. Interposition of lung tissue between the inferior surface of the heart and the diaphragm (arrow), typically seen in congenital absence of the pericardium as a result of the unusually posterior position of the heart. Image acquired with a cardiac-gated HASTE sequence as detailed in Figure 2A.Both displacement of the heart and interposition of lung parenchyma between heart and diaphragm are typical features of congenital absence of the pericardium, and herniation of the left ventricular apex suggests partial absence of the pericardium distal to the indentation of the left ventricular wall. Because of the severity of tricuspid regurgitation, the patient underwent tricuspid valve surgery, and at operation the partial absence of the pericardium was confirmed. The pericardial defect was repaired with a Goretex patch, and the tricuspid valve was replaced with a 33-mm biological (Perimount) prosthesis because a large defect in the anterior leaflet rendered it unsuitable for repair. Postoperative CMR (Figure 4) demonstrated successful restoration of normal left ventricular anatomy and normalization of right ventricular volumes (end-diastolic volume, 176 mL) with only mild tricuspid regurgitation. The patient recovered quickly from surgery and remains well. Download figureDownload PowerPointFigure 4. Restoration of the left ventricular anatomy in the same view as in Figure 2B. Note the considerable artifact from the metal frame of the tricuspid valve prosthesis (*). Image acquired with cardiac-gated TrueFISP cine sequence as detailed in Figure 2B.Lateral and posterior displacement of the heart and interposition of lung parenchyma between heart and diaphragm are typical features of congenital absence of the pericardium.1–3 Although CMR is useful in determination of anatomic abnormalities, the lack of visibility of the pericardium on CMR images does not prove its congenital absence and may lead to an erroneous diagnosis of pericardial absence in up to 10% of patients.4 In the present case, the features of left ventricular indentation, cardiac displacement, and lung interposition between heart and diaphragm rather than direct visualization of the pericardium suggested the diagnosis, which was confirmed by surgery. In patients with absence of the pericardium, ECG often shows incomplete right bundle-branch block with poor R-wave progression as a result of the leftward displacement of the heart,4,5 as seen in the present case. Congenital absence of the pericardium is a rare condition, and patients may either be asymptomatic or present with nonexertional stabbing chest pains. Approximately half the cases of congenital absence of the pericardium are associated with dilatation of the right ventricle, and other congenital cardiovascular and pulmonary abnormalities may be present.5 The male to female ratio has been reported as 3:1.5 Prognosis may be benign,1 particularly in complete absence of the pericardium, but has not been adequately clarified. Surgical pericardioplasty (Goretex mesh) may only be considered for highly symptomatic patients1 or in those patients who present with partial absence that leads to herniation or strangulation of cardiac and vascular structures.The online-only Data Supplement, consisting of a movie, can be found at http://circ.ahajournals.org/cgi/content/full/116/6/e126/DC1.DisclosuresNone.FootnotesCorrespondence to Dr Michaela Scheuermann-Freestone, Oxford Centre for Clinical Magnetic Resonance Research, John Radcliffe Hospital, Headley Way, Oxford OX3 9DU, United Kingdom. Email [email protected]References1 Gatzoulis MA, Mink MD, Merchant N, Van Arsdell GS, McCrindle BW, Webb GD. Isolated congenital absence of the pericardium: clinical presentation, diagnosis and management. Ann Thorac Surg. 2000; 69: 1209–1215.CrossrefMedlineGoogle Scholar2 Raman SV, Daniels CJ, Katz SE, Ryan JM, King MA. Congenital absence of the pericardium. Circulation. 2001; 104: 1447–1448.CrossrefMedlineGoogle Scholar3 Ratib O, Perloff JK, Williams WG. Congenital complete absence of the pericardium. Circulation. 2001; 103: 3154–3155.CrossrefMedlineGoogle Scholar4 Abbas AE, Appleton CP, Liu PT, Sweeney JP. Congenital absence of the pericardium: case presentation and review of literature. Int J Cardiol. 2005; 98: 21–25.CrossrefMedlineGoogle Scholar5 Gehlmann HR, Van Ingen GJ. Symptomatic congenital complete absence of the left pericardium: case report and review of the literature. Eur Heart J. 1989; 10: 670–675.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Terry N, Manapragada P, Aziz M and Singh S (2021) Review of pericardial disease on computed tomography, Journal of Medical Imaging and Radiation Sciences, 10.1016/j.jmir.2021.09.005, 52:3, (S65-S77), Online publication date: 1-Nov-2021. Abou Hassan O and Kwon D (2020) Update on MRI Techniques for Evaluation of Pericardial Disease, Current Cardiology Reports, 10.1007/s11886-020-01392-0, 22:11, Online publication date: 1-Nov-2020. Kligerman S (2019) Imaging of Pericardial Disease, Radiologic Clinics of North America, 10.1016/j.rcl.2018.09.001, 57:1, (179-199), Online publication date: 1-Jan-2019. Foo J, Koh C, Sahlén A, Tang H and Lim C (2018) Congenital Partial Absence of Pericardium: A Mimic of Arrhythmogenic Right Ventricular Cardiomyopathy, Case Reports in Medicine, 10.1155/2018/4297280, 2018, (1-4), . Lopez D and Asher C (2017) Congenital Absence of the Pericardium, Progress in Cardiovascular Diseases, 10.1016/j.pcad.2016.12.002, 59:4, (398-406), Online publication date: 1-Jan-2017. Parmar Y, Shah A, Poon M and Kronzon I (2017) Congenital Abnormalities of the Pericardium, Cardiology Clinics, 10.1016/j.ccl.2017.07.012, 35:4, (601-614), Online publication date: 1-Nov-2017. Macaione F, Barison A, Pescetelli I, Pali F, Pizzino F, Terrizzi A, Di Lisi D, Novo G, Todiere G, Assennato P, Novo S and Aquaro G (2016) Quantitative criteria for the diagnosis of the congenital absence of pericardium by cardiac magnetic resonance, European Journal of Radiology, 10.1016/j.ejrad.2015.12.021, 85:3, (616-624), Online publication date: 1-Mar-2016. Cook T (2015) Congenital absence of the pericardium Pearls and Pitfalls in Cardiovascular Imaging, 10.1017/CBO9781139152228.023, (72-73) Bogaert J and Francone M (2013) Pericardial Disease: Value of CT and MR Imaging, Radiology, 10.1148/radiol.13121059, 267:2, (340-356), Online publication date: 1-May-2013. Steinberg C, Pelletier M and Champagne J (2012) Complete Absence of Precordial R Waves Due to Absence of Left-Sided Pericardium, Annals of Noninvasive Electrocardiology, 10.1111/j.1542-474X.2012.00507.x, 17:4, (401-404), Online publication date: 1-Oct-2012. 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Bogaert J and Francone M (2009) Cardiovascular magnetic resonance in pericardial diseases, Journal of Cardiovascular Magnetic Resonance, 10.1186/1532-429X-11-14, 11:1, Online publication date: 1-Dec-2009. 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. Nassiri N, Yu A, Statkus N and Gosselin M (2009) Imaging of Cardiac Herniation in Traumatic Pericardial Rupture, Journal of Thoracic Imaging, 10.1097/RTI.0b013e31818d12de, 24:1, (69-72), Online publication date: 1-Feb-2009. Related articlesCorrectionCirculation. 2011;124:e301-e301 August 7, 2007Vol 116, Issue 6 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.107.701599PMID: 17679622 Originally publishedAugust 7, 2007 PDF download Advertisement SubjectsComputerized Tomography (CT)Congenital Heart DiseaseDevelopmental Biology
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