Opinion statement
Cardiac sarcoidosis can be life threatening if not promptly diagnosed and treated appropriately and aggressively. The diagnosis of myocardial disease is often difficult and circumstantial because there are no reliable diagnostic tests. Except for the finding of noncaseating granulomas on endomyocardial biopsy, most tests are limited and nonspecific. Therefore, the decision of initiating treatment is based on the patient’s symptoms and the course of the disease, rather than on the presence of histologic confirmation. The goal of therapy is to prevent irreversible cardiomyopathy and to thwart the progression to heart transplantation. The mainstay of treatment is corticosteroids, although there are no large randomized trials analyzing corticosteroid use. The combination with other immunosuppressant agents, such as Methotrexate and Azathioprine, is initiated on the patient’s failing or experiencing severe side effects from corticosteroids. While there are small studies proving the efficacy of tumor necrosis factor-inhibitors in cardiac sarcoidosis, more experience with these agents is needed. Catheter ablation or placement of implantable devices is indicated prophylactically in patients with severe ventricular tachyarrhythmias. Heart transplantation should be considered in patients with severe heart failure refractory to medical therapy. This article focuses on the current diagnostic tests and treatment recommendations for cardiac sarcoidosis.
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The pathology of pulmonary sarcoidosis: update. Ma Y, Gal A, Koss MN. Semin Diagn Pathol. 2007 Aug;24(3):150–61. Review. PMID: 17882899 [PubMed - indexed for MEDLINE]
Chapelon-Abric C, de Zuttere D, Duhaut P, et al. Cardiac sarcoidosis: a retrospective study of 41 cases. Medicine (Baltimore). 2004;83:315–34.
Kim JS, Judson MA, Donnino R, et al. Cardiac sarcoidosis. Am Heart J. 2009;157:9.
Thomsen TK, Eriksson T. Myocardial sarcoidosis in forensic medicine. Am J Forensic Med Pathol. 1999;20:52.
Sharma OP, Maheshwari A, Thaker K. Myocardial sarcoidosis. Chest. 1993;103:253–8.
Matsui Y, Iwai K, Tachibana T, et al. Clinicopathological study on fatal myocardial sarcoidosis. Ann N Y Acad Sci. 1976;278:455–69.
Iwai K, Sekiguti M, Hosoda Y, et al. Racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis. 1994;11:26–31.
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;119:167–72.
Fleming HA. Sarcoid heart disease. Br Heart J 1974;36:54–68.
Roberts WC, McAllister Jr HA, Ferrans VJ. Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (group 1) and review of 78 previously described necropsy patients (group 11). Am J Med. 1977;63:86–108.
Angomachalelis N, Hourzamanis A, Salem N, et al. Pericardial effusion concomitant with specific heart musle disease in systemic sarcoidosis. Postgrad Med J. 1994;70 Suppl 1:S8–S12.
Garret J, O’Neill H, Blake S. Constrictive pericarditis associated with sarcoidosis. Am Heart J. 1984;107:394.
Israel RH, Poe RH. Massive pericardial effusion in sarcoidosis. Respiration. 1994;61:176–80.
Verkleeren JL, Glover MU, Bloor C, et al. Cardiac tamponade secondary to sarcoidosis. Am Heart J. 1983;106:601–3.
Shorr AF, Helman DL, Davies DB, Nathan SD. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur Respir J. 2005;25:783–8.
Handa T, Uozato H, Higa R, et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest. 2006;129:1246–52.
Nunes H, Humbert M, Capron F, et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax. 2006;61:68–74.
Hiraga, H, Yuwai, K, Hiroe, M, et al. Guidelines for the diagnosis of cardiac sarcoidosis: Study Report of Diffuse Pulmonary Diseases. The Japanese Ministry of Health and Welfare 1993; pp. 23–24
Smedema JP, Snoep G, van Kroonenburgh MP, et al. Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis. J Am Coll Cardiol. 2005;45:1683.
Uemura A, Morimoto S, Hiramitsu S, et al. Histologic diagnostic rate of cardiac sarcoidosis: evaluation of endomyocardial biopsies. Am Heart J. 1999;138:299–302.
Ratner SJ, Fenoglio JJ, Ursell P. Utility of endomyocardial biopsy in the diagnosis of cardiac sarcoidosis. Chest. 1986;90:528–33.
Silverman KJ, Hutchins GM, Buckley BH. Cardiac Sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation. 1978;58:1204–11.
Suzuki T, Kanda T, Kubota S, et al. Holter monitoring as a noninvasive indicator of cardiac involvement in sarcoidosis. Chest. 1994;106:1021.
Kinney EL, Jackson GL, Reeves WC, et al. Thallium-scan myocardial defects and echocardiographic abnormalities in patients with sarcoidosis without clinical cardiac dysfunction: an analysis of 44 patients. Am J Med. 1980;68:497–503.
Fahy GJ, Marwick T, McCreery CJ, et al. Doppler echocardiographic detection of left ventricular diastolic dysfunction in patients with pulmonary sarcoidosis. Chest. 1996;109:62–6.
Burstow DJ, Tajik AJ, Bailey KR, et al. Two-dimensional echocardiographic findings in systemic sarcoidosis. Am J Cardiol. 1989;63:478–82.
Lewin RF, Mor R, Spitzer S, et al. Echocardiographic evaluation of patients with systemic sarcoidosis. Am Heart J. 1985;110:116–22.
Bulkley BH, Rouleau JR, Whitaker JQ, et al. The use of 201thallium for myocardial perfusion imaging in sarcoid heart disease. Chest. 1977;72:27–32.
Haywood LJ, Sharma OP, Siegel ME, et al. Detection of myocardial sarcoidosis by thallium-201 imaging. J Natl Med Assoc. 1982;74:959.
Tellier P, Paycha F, Antony I, et al. Reversibility by dipyridamole of thallium-201 myocardial defects in patients with sarcoidosis. Am J Med. 1988;85:189–93.
Mañá J. Nuclear imaging. 67Gallium, 201thallium, 18F-labeled fluoro-2-deoxy-D-glucose positron emission tomography. Clin Chest Med. 1997;18:799.
Le Guludec D, Menad F, Faraggi M, et al. Myocardial sarcoidosis: clinical value of technetium-99m sestamibi tomoscintigraphy. Chest. 1994;106:1675–168.
Eguchi M, Tsuchihashi K, Hotta D, et al. Technetium 99m sestamibi/tetrofosmin myocardial perfusion scanning in cardiac and noncardiac sarcoidosis. Cardiology. 2000;94:193–9.
Okayama K, Kurata C, Tawarahara K, et al. Diagnostic and prognostic value of myocardial scintigraphy with thallium-201 and gallium-67 in cardiac sarcoidosis. Chest. 1995;107:330.
Kiuchi S, Teraoka K, Koizumi K, et al. Usefulness of late gadolinium enhancement combined with MRI and 67-Ga scintigraphy in the diagnosis of cardiac sarcoidosis and disease activity evaluation. Int J Cardiovasc Imaging. 2007;23:237.
Tadamura E, Yamamuro M, Kubo S, et al. Effectiveness of delayed enhanced MRI for identification of cardiac sarcoidosis: comparison with radionuclide imaging. AJR Am J Roentgenol. 2005;185:110–5.
Shimada T, Shimada K, Sakane T, et al. Diagnosis of cardiac sarcoidosis and evaluation of the effects of steroid therapy by gadolinium-DTPA-enhanced magnetic resonance imaging. Am J Med. 2001;110:520.
Matsuki M, Matsuo M. MR findings of myocardial sarcoidosis. Clin Radiol 2000;55:323–5.
Smedema JP, Snoep G, van Kroonenburgh MP, et al. The additional value of gadolinium-enhanced MRI to standard assessment for cardiac involvement in patients with pulmonary sarcoidosis. Chest. 2005;128:1629.
Vignaux O, Dhote R, Duboc D, et al. Clinical significance of myocardial magnetic resonance abnormalities in patients with sarcoidosis: a 1-year follow-up study. Chest. 2002;122:1895.
Patel MR, Cawley PJ, Heitner JF, et al. Detection of myocardial damage in patients with sarcoidosis. Circulation. 2009;120:1969.
Yamagishi H, Shirai N, Takagi M, et al. Identification of cardiac sarcoidosis with (13)N-NH(3)/(18)F-FDG PET. J Nucl Med. 2003;44:1030–6.
Butany J, Bahl NE, Morales K, et al. The intricacies of cardiac sarcoidosis: a case report involving the coronary arteries and a review of the literature. Cardiovasc Pathol. 2006;15:222.
Takada K, Ina Y, Yamamoto M, et al. Prognosis after pacemaker implantation in cardiac sarcoidosis in Japan. Clinical evaluation of corticosteroid therapy. Sarcoidosis. 1994;11:113.
Yazaki Y, Isobe M, Hiroe M, et al. Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Cardiol. 2001;88:1006.
du Bois RM. Corticosteroids in sarcoidosis: friend or foe? Eur Respir J. 1994;7:1203.
Baughman RP, Sharma OP, Lynch III JP. Sarcoidosis: is therapy effective? Semin Respir Infect. 1998;13:255–73.x
Lower EE, Baughman RP. Prolonged use of methotrexate for sarcoidosis. Arch Intern Med. 1995;155:846–51. doi:10.1001/archinte.155.8.846.
Webster GF, Razsi LK, Sanchez M, et al. Weekly low-dose methotrexate therapy for cutaneous sarcoidosis. J Am Acad Dermatol. 1991;24:451–4.
Baughman RP, Lower EE. A clinical approach to the use of methotrexate for sarcoidosis. Thorax. 1999;54:742–6.
Pacheco Y, Marechal C, Marechal F, et al. Azathioprine treatment of chronic pulmonary sarcoidosis. Sarcoidosis. 1985;2:107.
Müller-Quernheim J, Kienast K, Held M, et al. Treatment of chronic sarcoidosis with an azathioprine/prednisolone regimen. Eur Respir J. 1999;14:1117.
Baughman RP, Lower EE. Infliximab for refractory sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2001;18:70.
Baughman RP, Drent M, Kavuru M, et al. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med. 2006;174:795.
Rossman MD, Newman LS, Baughman RP, et al. A double-blinded, randomized, placebo-controlled trial of infliximab in subjects with active pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23:201.
Doty JD, Mazure JE, Judson MA. Treatment of sarcoidosis with infliximab. Chest. 2005;127:1064–71.
Roberts SD, Wilkes DS, Burgett RA, Knox KS. Refractory sarcoidosis responding to infliximab. Chest. 2003;124:2028–31.
Uthman I, Touma Z, Khoury M. Cardiac sarcoidosis responding to monotherapy with infliximab. Clin Rheumatol. 2007;26:2001–3.
Shammas RL, Movahed A. Sarcoidosis of the heart. Clin Cardiol. 1993;16:462–72.
Ahmad K, Kim YH, Spitzer AR, et al. Total nodal radiation in progressive sarcoidosis. Case report Am J Clin Oncol. 1992;15:311.
Jefic D, Joel B, Good E, et al. Role of radiofrequency catheter ablation of ventricular tachycardia in cardiac sarcoidosis: report from a multicenter registry. Heart Rhythm. 2009;6:189.
Bohnen M, Stevenson WG, Tedrow UB, et al. Incidence and predictors of major complications from contemporary catheter ablation to treat cardiac arrhythmias. Heart Rhythm. 2011;8(11):1661.
Kay GN, Epstein AE, Dailey SM, Plumb VJ. Role of radiofrequency ablation in the management of supraventricular arrhythmias: experience in 760 consecutive patients. J Cardiovasc Electrophysiol. 1993;4(4):371.
Chen SA, Chiang CE, Tai CT, et al. Complications of diagnostic electrophysiologic studies and radiofrequency catheter ablation in patients with tachyarrhythmias: an eight-year survey of 3,966 consecutive procedures in a tertiary referral center. Am J Cardiol. 1996;77(1):41.
Scheinman MM, Huang S. The 1998 NASPE prospective catheter ablation registry. Pacing Clin Electrophysiol. 2000;23(6):1020.
Bubien RS, Knotts-Dolson SM, Plumb VJ, Kay GN. Effect of radiofrequency catheter ablation on health-related quality of life and activities of daily living in patients with recurrent arrhythmias. Circulation. 1996;94:1585–91.
Strickberger SA, Man KC, Daoud EG, et al. A prospective evaluation of catheter ablation of ventricular tachycardia as adjuvant therapy in patients with coronary artery disease and an implantable cardioverter-defibrillator. Circulation. 1997;96:1525–31.
Crandall MA, Bradley DJ, Packer DL, Asirvatham SJ. Contemporary management of atrial fibrillation: update on anticoagulation and invasive management strategies. Mayo Clin Proc. 2009;84:643–62.
Winters SL, Cohen M, Greenberg S, et al. Sustained ventricular tachycardia associated with sarcoidosis: assessment of the underlying cardiac anatomy and the prospective utility of programmed ventricular stimulation, drug therapy and an implantable antitachycardia device. J Am Coll Cardiol. 1991;18:937.
Bajaj AK, Kopelman HA, Echt DS. Cardiac sarcoidosis with sudden death: treatment with the automatic implantable cardioverter defibrillator. Am Heart J. 1988;116:557.
Epstein AE, DiMarco JP, Ellenbogen KA, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. J Am Coll Cardiol. 2008;51:e1.
Wood MA, Ellenbogen KA. Cardiology patient pages. Cardiac pacemakers from the patient’s perspective. Circulation. 2002;105(18):2136.
Zaidi AR, Zaidi A, Vaitkus PT. Outcome of heart transplantation in patients with sarcoid cardiomyopathy. J Heart Lung Transplant. 2007;26:714.
Valantine HA, Tazelaar HD, Macoviak J, et al. Cardiac sarcoidosis: response to steroids and transplantation. J Heart Transplant. 1987;6:244–50.
Oni AA, Hershberger RE, Norman DJ, et al. Recurrence of sarcoidosis in a cardiac allograft: control with augmented corticosteroids. J Heart Lung Transplant. 1992;11:367.
Taylor DO, Stehlik J, Edwards LB, et al. Registry of the International Society for Heart and Lung Transplantation: Twenty-sixth Official Adult Heart Transplant Report-2009. J Heart Lung Transplant. 2009;28(10):1007.
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Bussinguer, M., Danielian, A. & Sharma, O.P. Cardiac Sarcoidosis: Diagnosis and Management. Curr Treat Options Cardio Med 14, 652–664 (2012). https://doi.org/10.1007/s11936-012-0208-3
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DOI: https://doi.org/10.1007/s11936-012-0208-3