Journal of Nuclear Cardiology

, Volume 25, Issue 2, pp 443–456 | Cite as

A case of longitudinal care of a patient with cardiac sarcoidosis

  • Rayan Saab
  • Pradeep Bhambhvani
  • Ami E. Iskandrian
  • Fadi G. Hage
Case Presentation Corner


Cardiac sarcoidosis has long been an evasive diagnosis with a spectrum of clinical presentations that extend from asymptomatic to ventricular arrhythmias and sudden cardiac death. The diagnosis has traditionally relied on histology which suffers from the low sensitivity of endomyocardial biopsy due to the patchy nature of the disease in addition to its invasive nature. Due to significant advancements in imaging, it is now possible to accurately identify cardiac sarcoidosis using non-invasive imaging modalities even without histological confirmation. Emerging guidelines are highlighting the role of multimodality imaging in the diagnosis and management of this challenging entity. We present the case of a 36-year-old man known to have sarcoidosis in which a variety of imaging modalities not only assisted in the diagnosis of cardiac sarcoidosis, but also played a key role in the monitoring of disease activity and response to therapy.


PET/CT imaging Magnetic resonance imaging Radiopharmaceuticals Inflammation Diagnostic and prognostic applications 



Cardiac sarcoidosis


Transthoracic echocardiogram


Endomyocardial biopsy


Single-photon emission tomography


Myocardial perfusion imaging




Positron emission tomography


Cardiac magnetic resonance imaging


Late gadolinium enhancement


Cardiac computer tomography angiography



Dr. Hage has received research grant support from Astellas Pharma. Drs. Bhambhvani, Iskandrian and Saab have no conflicts.


  1. 1.
    Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation. 1978;58:1204–11.CrossRefPubMedGoogle Scholar
  2. 2.
    Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357:2153–65.CrossRefPubMedGoogle Scholar
  3. 3.
    Rybicki BA, Major M, Popovich J Jr, Maliarik MJ, Iannuzzi MC. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol. 1997;145:234–41.CrossRefPubMedGoogle Scholar
  4. 4.
    Bresnitz EA, Strom BL. Epidemiology of sarcoidosis. Epidemiol Rev. 1983;5:124–56.CrossRefPubMedGoogle Scholar
  5. 5.
    Gideon NM, Mannino DM. Sarcoidosis mortality in the United States 1979-1991: an analysis of multiple-cause mortality data. Am J Med. 1996;100:423–7.CrossRefPubMedGoogle Scholar
  6. 6.
    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.PubMedGoogle Scholar
  7. 7.
    Nery PB, Keren A, Healey J, Leug E, Beanlands RS, Birnie DH. Isolated cardiac sarcoidosis: establishing the diagnosis with electroanatomic mapping-guided endomyocardial biopsy. Can J Cardiol. 1015;2013(29):e1–3.Google Scholar
  8. 8.
    Yazaki Y, Isobe M, Hiroe M, Morimoto S, Hiramitsu S, Nakano T, et al. Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Cardiol. 2001;88:1006–10.CrossRefPubMedGoogle Scholar
  9. 9.
    Iwai K, Sekiguti M, Hosoda Y, DeRemee RA, Tazelaar HD, Sharma OP, et al. Racial difference in cardiac sarcoidosis incidence observed at autopsy. Sarcoidosis. 1994;11:26–31.PubMedGoogle Scholar
  10. 10.
    Boglioli LR, Taff ML, Funke S, Mihalakis I. Sudden death due to sarcoid heart disease. J Forensic Sci. 1998;43:1072–3.CrossRefPubMedGoogle Scholar
  11. 11.
    Nery PB, Beanlands RS, Nair GM, Green M, Yang J, McArdle BA, et al. Atrioventricular block as the initial manifestation of cardiac sarcoidosis in middle-aged adults. J Cardiovasc Electrophysiol. 2014;25:875–81.CrossRefPubMedGoogle Scholar
  12. 12.
    Roberts WC, McAllister HA Jr, 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.CrossRefPubMedGoogle Scholar
  13. 13.
    Doughan AR, Williams BR. Cardiac sarcoidosis. Heart. 2006;92:282–8.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Cooper LT, Baughman KL, Feldman AM, Frustaci A, Jessup M, Kuhl U, et al. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology. J Am Coll Cardiol. 2007;50:1914–31.CrossRefPubMedGoogle Scholar
  15. 15.
    Erthal F, Juneau D, Lim SP, Dwivedi G, Nery PB, Birnie D, et al. Imaging of cardiac sarcoidosis. Q J Nucl Med Mol Imaging. 2016;60:252–63.PubMedGoogle Scholar
  16. 16.
    Birnie DH, Sauer WH, Bogun F, Cooper JM, Culver DA, Duvernoy CS, et al. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart Rhythm. 2014;11:1305–23.CrossRefPubMedGoogle Scholar
  17. 17.
    Terasaki Fumio, Yoshinaga Keiichiro. New guidelines for diagnosis of cardiac sarcoidosis in Japan. Ann Nucl Cardiol. 2017. Scholar
  18. 18.
    Sadek MM, Yung D, Birnie DH, Beanlands RS, Nery PB. Corticosteroid therapy for cardiac sarcoidosis: a systematic review. Can J Cardiol. 2013;29:1034–41.CrossRefPubMedGoogle Scholar
  19. 19.
    Hulten E, Aslam S, Osborne M, Abbasi S, Bittencourt MS, Blankstein R. Cardiac sarcoidosis-state of the art review. Cardiovasc Diagn Ther. 2016;6:50–63.PubMedPubMedCentralGoogle Scholar
  20. 20.
    Ayoub C, Pena E, Ohira H, Dick A, Leung E, Nery PB, et al. Advanced imaging of cardiac sarcoidosis. Curr Cardiol Rep. 2015;17:17.CrossRefPubMedGoogle Scholar
  21. 21.
    Slart R, Glaudemans A, Lancellotti P, Hyafil F, Blankstein R, Schwartz RG, et al. A joint procedural position statement on imaging in cardiac sarcoidosis: from the Cardiovascular and inflammation & infection committees of the european association of nuclear medicine, the European association of cardiovascular imaging, and the American Society of nuclear cardiology. J Nucl Cardiol. 2017;8(10):1073–89. Scholar
  22. 22.
    Nagano N, Nagai T, Sugano Y, Morita Y, Asaumi Y, Aiba T, et al. Association between basal thinning of interventricular septum and adverse long-term clinical outcomes in patients with cardiac sarcoidosis. Circ J. 2015;79:1601–8.CrossRefPubMedGoogle Scholar
  23. 23.
    Pizarro C, Kluenker F, Hammerstingl C, Skowasch D. Diagnostic value of speckle-tracking echocardiography in confirmed cardiac sarcoidosis. Clin Res Cardiol. 2016;105:884–6.CrossRefPubMedGoogle Scholar
  24. 24.
    Schouver ED, Moceri P, Doyen D, Tieulie N, Queyrel V, Baudouy D, et al. Early detection of cardiac involvement in sarcoidosis with 2-dimensional speckle-tracking echocardiography. Int J Cardiol. 2017;227:711–6.CrossRefPubMedGoogle Scholar
  25. 25.
    Murtagh G, Laffin LJ, Patel KV, Patel AV, Bonham CA, Yu Z, et al. Improved detection of myocardial damage in sarcoidosis using longitudinal strain in patients with preserved left ventricular ejection fraction. Echocardiography. 2016;33:1344–52.CrossRefPubMedPubMedCentralGoogle Scholar
  26. 26.
    Patel MB, Mor-Avi V, Murtagh G, Bonham CA, Laffin LJ, Hogarth DK, et al. Right heart involvement in patients with sarcoidosis. Echocardiography. 2016;33:734–41.CrossRefPubMedGoogle Scholar
  27. 27.
    Kouranos V, Wells AU, Sharma R, Underwood SR, Wechalekar K. Advances in radionuclide imaging of cardiac sarcoidosis. Br Med Bull. 2015;115:151–63.CrossRefPubMedGoogle Scholar
  28. 28.
    Momose M, Fukushima K, Kondo C, Serizawa N, Suzuki A, Abe K, et al. Diagnosis and detection of myocardial injury in active cardiac sarcoidosis-significance of myocardial fatty acid metabolism and myocardial perfusion mismatch. Circ J. 2015;79:2669–76.CrossRefPubMedGoogle Scholar
  29. 29.
    Blankstein R, Osborne M, Naya M, Waller A, Kim CK, Murthy VL, et al. Cardiac positron emission tomography enhances prognostic assessments of patients with suspected cardiac sarcoidosis. J Am Coll Cardiol. 2014;63:329–36.CrossRefPubMedGoogle Scholar
  30. 30.
    Boellaard R, Delgado-Bolton R, Oyen WJ, Giammarile F, Tatsch K, Eschner W, et al. FDG PET/CT: eANM procedure guidelines for tumour imaging: version 2.0. Eur J Nucl Med Mol Imaging. 2015;42:328–54.CrossRefPubMedGoogle Scholar
  31. 31.
    Lu Y, Grant C, Xie K, Sweiss NJ. Suppression of myocardial 18f-fdg uptake through prolonged high-fat, high-protein, and very-low-carbohydrate diet before fdg-pet/ct for evaluation of patients with suspected cardiac sarcoidosis. Clin Nucl Med. 2017;42:88–94.CrossRefPubMedGoogle Scholar
  32. 32.
    Cheng VY, Slomka PJ, Ahlen M, Thomson LE, Waxman AD, Berman DS. Impact of carbohydrate restriction with and without fatty acid loading on myocardial 18F-FDG uptake during PET: a randomized controlled trial. J Nucl Cardiol. 2010;17:286–91.CrossRefPubMedGoogle Scholar
  33. 33.
    Waller AH, Blankstein R. Quantifying myocardial inflammation using F18-fluorodeoxyglucose positron emission tomography in cardiac sarcoidosis. J Nucl Cardiol. 2014;21:940–3.CrossRefPubMedGoogle Scholar
  34. 34.
    Chareonthaitawee P, Beanlands RS, Chen W, Dorbala S, Miller EJ, Murthy VL, et al. Joint SNMMI-ASNC expert consensus document on the role of (18)F-FDG PET/CT in cardiac sarcoid detection and therapy monitoring. J Nucl Cardiol. 2017;24:1741–58.CrossRefPubMedGoogle Scholar
  35. 35.
    Okumura W, Iwasaki T, Toyama T, Iso T, Arai M, Oriuchi N, et al. Usefulness of fasting 18F-FDG PET in identification of cardiac sarcoidosis. J Nucl Med. 2004;45:1989–98.PubMedGoogle Scholar
  36. 36.
    Skali H, Schulman AR, Dorbala S. 18F-FDG PET/CT for the assessment of myocardial sarcoidosis. Curr Cardiol Rep. 2013;15:352.CrossRefPubMedCentralGoogle Scholar
  37. 37.
    Yamagishi H, Shirai N, Takagi M, Yoshiyama M, Akioka K, Takeuchi K, et al. Identification of cardiac sarcoidosis with (13)N-NH(3)/(18)F-FDG PET. J Nucl Med. 2003;44:1030–6.PubMedGoogle Scholar
  38. 38.
    Osborne MT, Hulten EA, Singh A, Waller AH, Bittencourt MS, Stewart GC, et al. Reduction in (1)(8)F-fluorodeoxyglucose uptake on serial cardiac positron emission tomography is associated with improved left ventricular ejection fraction in patients with cardiac sarcoidosis. J Nucl Cardiol. 2014;21:166–74.CrossRefPubMedGoogle Scholar
  39. 39.
    Kruse MJ, Kovell L, Kasper EK, Pomper MG, Moller DR, Solnes L, et al. Myocardial blood flow and inflammatory cardiac sarcoidosis. JACC Cardiovasc Imaging. 2017;10:157–67.CrossRefPubMedGoogle Scholar
  40. 40.
    Norikane T, Yamamoto Y, Maeda Y, Noma T, Nishiyama Y. 18F-FLT PET imaging in a patient with sarcoidosis with cardiac involvement. Clin Nucl Med. 2015;40:433–4.CrossRefPubMedGoogle Scholar
  41. 41.
    Patel MR, Cawley PJ, Heitner JF, Klem I, Parker MA, Jaroudi WA, et al. Detection of myocardial damage in patients with sarcoidosis. Circulation. 2009;120:1969–77.CrossRefPubMedPubMedCentralGoogle Scholar
  42. 42.
    Ohira H, Tsujino I, Ishimaru S, Oyama N, Takei T, Tsukamoto E, et al. Myocardial imaging with 18F-fluoro-2-deoxyglucose positron emission tomography and magnetic resonance imaging in sarcoidosis. Eur J Nucl Med Mol Imaging. 2008;35:933–41.CrossRefPubMedGoogle Scholar
  43. 43.
    Smedema JP, Snoep G, van Kroonenburgh MP, van Geuns RJ, Dassen WR, Gorgels AP, et al. Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis. J Am Coll Cardiol. 2005;45:1683–90.CrossRefPubMedGoogle Scholar
  44. 44.
    Hulten E, Agarwal V, Cahill M, Cole G, Vita T, Parrish S, et al. Presence of late gadolinium enhancement by cardiac magnetic resonance among patients with suspected cardiac sarcoidosis is associated with adverse cardiovascular prognosis: a systematic review and meta-analysis. Circ Cardiovasc Imaging. 2016;9:e005001.CrossRefPubMedPubMedCentralGoogle Scholar
  45. 45.
    Greulich S, Kitterer D, Latus J, Aguor E, Steubing H, Kaesemann P, et al. Comprehensive cardiovascular magnetic resonance assessment in patients with sarcoidosis and preserved left ventricular ejection fraction. Circ Cardiovasc Imaging. 2016;9:PMID: 27903537.Google Scholar
  46. 46.
    Puntmann VO, Isted A, Hinojar R, Foote L, Carr-White G, Nagel E. T1 and T2 mapping in recognition of early cardiac involvement in systemic sarcoidosis. Radiology. 2017;285:63–72.CrossRefPubMedGoogle Scholar
  47. 47.
    Dweck MR, Abgral R, Trivieri MG, Robson PM, Karakatsanis N, Mani V, et al. Hybrid magnetic resonance imaging and positron emission tomography with fluorodeoxyglucose to diagnose active cardiac sarcoidosis. JACC Cardiovasc Imaging. 2018;11:94–107.CrossRefPubMedGoogle Scholar
  48. 48.
    Kamphuis LS, Kwekkeboom DJ, Missotten TO, Baarsma GS, Dalm VA, Dik WA, et al. Somatostatin receptor scintigraphy patterns in patients with sarcoidosis. Clin Nucl Med. 2015;40:925–9.CrossRefPubMedGoogle Scholar
  49. 49.
    Reiter T, Werner RA, Bauer WR, Lapa C. Detection of cardiac sarcoidosis by macrophage-directed somatostatin receptor 2-based positron emission tomography/computed tomography. Eur Heart J. 2015;36:2404.CrossRefPubMedGoogle Scholar
  50. 50.
    Vis R, Malviya G, Signore A, Grutters JC, Meek B, van de Garde EM, et al. (9)(9)mTc-anti-TNF-alpha antibody for the imaging of disease activity in pulmonary sarcoidosis. Eur Respir J. 2016;47:1198–207.CrossRefPubMedGoogle Scholar

Copyright information

© American Society of Nuclear Cardiology 2018

Authors and Affiliations

  • Rayan Saab
    • 1
  • Pradeep Bhambhvani
    • 2
  • Ami E. Iskandrian
    • 1
  • Fadi G. Hage
    • 1
    • 3
  1. 1.Division of Cardiovascular Disease, Department of MedicineUniversity of Alabama at BirminghamBirminghamUSA
  2. 2.Division of Molecular Imaging and Therapeutics, Department of RadiologyUniversity of Alabama at BirminghamBirminghamUSA
  3. 3.Section of CardiologyBirmingham Veterans Affairs Medical CenterBirminghamUSA

Personalised recommendations