Coronary Artery Vasculitis as a Presentation of Cardiac Sarcoidosis
2012; Lippincott Williams & Wilkins; Volume: 125; Issue: 6 Linguagem: Inglês
10.1161/circulationaha.110.990747
ISSN1524-4539
AutoresEmily Ward, Jose Nazari, Robert R. Edelman,
Tópico(s)Amyloidosis: Diagnosis, Treatment, Outcomes
ResumoHomeCirculationVol. 125, No. 6Coronary Artery Vasculitis as a Presentation of Cardiac Sarcoidosis Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessBrief ReportPDF/EPUBCoronary Artery Vasculitis as a Presentation of Cardiac Sarcoidosis Emily V. Ward, MD, Jose Nazari, MD and Robert R. Edelman, MD Emily V. WardEmily V. Ward From the Department of Radiology (E.V.W., R.R.E.) and Division of Cardiology (J.N.), NorthShore University HealthSystem, Evanston Hospital, Evanston, IL. , Jose NazariJose Nazari From the Department of Radiology (E.V.W., R.R.E.) and Division of Cardiology (J.N.), NorthShore University HealthSystem, Evanston Hospital, Evanston, IL. and Robert R. EdelmanRobert R. Edelman From the Department of Radiology (E.V.W., R.R.E.) and Division of Cardiology (J.N.), NorthShore University HealthSystem, Evanston Hospital, Evanston, IL. Originally published14 Feb 2012https://doi.org/10.1161/CIRCULATIONAHA.110.990747Circulation. 2012;125:e344–e346A 40-year-old white man was admitted for excision of a subcutaneous lesion on his torso. He reported a 12-month history of shortness of breath and exertional chest pain, both of which were relieved by rest. The patient was otherwise asymptomatic, and physical examination was noncontributory. He was not taking any medications. Electrocardiographic monitoring during the procedure revealed third-degree heart block with junctional escape and right bundle-branch block at 38 bpm (Figure 1). The patient was hemodynamically stable. An electrophysiology consultation was requested, and the patient was found to have an elevated serum angiotensin-converting enzyme level. Histological examination of the biopsy specimen showed noncaseating granulomata, which confirmed the diagnosis of sarcoidosis.Download figureDownload PowerPointFigure 1. Twelve-lead ECG taken 8 minutes after procedure while the patient was in recovery. This shows third-degree heart block with right bundle-branch block and a rate of 38 bpm.The patient had had an ungated noncontrast chest computed tomographic examination performed approximately 1 month earlier that showed mediastinal and hilar lymphadenopathy (Figure 2) along with multiple subcentimeter lung nodules (Figure 3). In retrospect, it demonstrated infiltration of the epicardial fat surrounding the right coronary artery within the atrioventricular groove (Figure 4).Download figureDownload PowerPointFigure 2. Noncontrast coronal computed tomography image (mediastinal windows) shows bilateral hilar and mediastinal lymphadenopathy (arrows).Download figureDownload PowerPointFigure 3. Noncontrast axial computed tomography image (lung windows) shows multiple lung nodules (arrows).Download figureDownload PowerPointFigure 4. Noncontrast axial computed tomography image (mediastinal windows) shows extensive infiltration of the epicardial fat within the right atrioventricular groove (arrow).A cardiac magnetic resonance study was performed that showed hypokinesis of the basal portions of the left ventricle and interventricular septum (Movie 1), with associated myocardial delayed enhancement (Figure 5). There was infiltration of the epicardial fat surrounding the right coronary artery (Figure 6). Less severe infiltration was present along the course of the left anterior descending coronary artery. There was thinning of the anteroseptal and apical portions of the left ventricular myocardium, but with relatively preserved systolic thickening (Movie 1). The patient was treated with oral prednisone 60 mg daily and implantation of a dual-chamber defibrillator.Download figureDownload PowerPointFigure 5. Short-axis delayed-enhancement image (acquired by single-shot inversion-recovery balanced steady-state gradient-echo acquisition) shows left ventricular myocardial enhancement (black arrow), thickening and delayed enhancement of the inferior interventricular groove (broken white arrow), and enhancement of the right ventricular myocardium (solid white arrow). RV indicates right ventricle; LV, left ventricle.Download figureDownload PowerPointFigure 6. Three-chamber true FISP (true fast imaging with steady state precession) magnetic resonance image shows extensive infiltration of the epicardial fat within the right atrioventricular groove. This T2-weighted image also demonstrates edema within the wall of the involved myocardium (lateral wall of the left ventricle and interventricular septum).Two months later, the patient presented for follow-up. He denied any symptoms at this time; however, an echocardiogram showed an ejection fraction of 30%. An ECG showed normal sinus rhythm with biventricular pacing. Cardiac catheterization was performed to exclude coronary artery disease; results were normal (Figures 7 and 8). The patient was told to continue taking antiarrhythmic medication and referred to the cardiac transplantation service.Download figureDownload PowerPointFigure 7. Image from cardiac catheterization showing a normal left main, left anterior descending, and left circumflex artery.Download figureDownload PowerPointFigure 8. Image from cardiac catheterization showing a normal right coronary artery.The incidence of cardiac involvement in patients with sarcoidosis is on the order of 76%, although it is frequently diagnosed postmortem.1 Epicardial coronary artery involvement is rare2; we are aware of only 1 biopsy-proven case of coronary sarcoidosis presenting as acute coronary syndrome.3 The present case is unusual in the severity and extent of infiltration of the epicardial fat surrounding the right coronary artery. Functional impairment due to presumed coronary vasculitis was evidenced by abnormal wall motion or thinning of the affected portions of the left ventricular myocardium.This case further broadens the spectrum of presentations of cardiac sarcoidosis and reiterates the importance of considering this disorder in the differential diagnosis of cardiac pathology that involves both the myocardium and coronary arteries.DisclosuresNone.Footnotes*Senior author.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.110.990747/-/DC1.Correspondence to Robert R. Edelman, MD, Department of Radiology, NorthShore University HealthSystem, Evanston Hospital, 2650 Ridge Ave, Evanston, IL 60201. E-mail [email protected]comReferences1. Perry A, Vuitch F. Causes of death in patients with sarcoidosis: a morphologic study of 38 autopsies with clinicopathologic correlations. Arch Pathol Lab Med. 1995; 119:167–172.MedlineGoogle Scholar2. Butany J, Bahl NE, Morales K, Thangaroopan M, Ross H, Rao V, Leong SW. The intricacies of cardiac sarcoidosis: a case report involving the coronary arteries and a review of the literature. Cardiovasc Pathol. 2006; 15:222–227.CrossrefMedlineGoogle Scholar3. Lam CS, Tolep KA, Metke MP, Glockner J, Cooper LT. Coronary sarcoidosis presenting as acute coronary syndrome. Clin Cardiol. 2009; 32:E68–E71.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Saric P, Young K, Rodriguez-Porcel M and Chareonthaitawee P (2021) PET Imaging in Cardiac Sarcoidosis: A Narrative Review with Focus on Novel PET Tracers, Pharmaceuticals, 10.3390/ph14121286, 14:12, (1286) Zureigat H, Frank R, Shah V, Makarenko V, Hucker W, Ho J, Wood M and Osborne M (2021) Cardiac Sarcoidosis Initially Diagnosed as Spontaneous Coronary Artery Dissection, JACC: Case Reports, 10.1016/j.jaccas.2021.05.017, 3:15, (1656-1660), Online publication date: 1-Nov-2021. 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A new entity identified by cardiovascular magnetic resonance and its clinical implications, International Journal of Cardiology, 10.1016/j.ijcard.2013.04.116, 168:3, (2971-2972), Online publication date: 1-Oct-2013. Chapelon-Abric C (2013) Cardiac sarcoidosis, Current Opinion in Pulmonary Medicine, 10.1097/MCP.0b013e32836436da, 19:5, (493-502), Online publication date: 1-Sep-2013. February 14, 2012Vol 125, Issue 6 Advertisement Article InformationMetrics © 2012 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.110.990747PMID: 22331924 Originally publishedFebruary 14, 2012 PDF download Advertisement SubjectsComputerized Tomography (CT)
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