Skip to main content

Advertisement

Log in

Echocardiography in Sarcoidosis

  • Echocardiography (JM Gardin and AH Waller, Section Editors)
  • Published:
Current Cardiology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Cardiac sarcoidosis (CS) is associated with significant morbidity and mortality. The diagnosis of CS is challenging and typically one that is only entertained after many other conditions have been ruled out. A high index of suspicion is necessary in order to correctly determine appropriate testing for the disease. Transthoracic echocardiography is the most readily available imaging modality available to help establish a diagnosis in a potential patient. However, no one echocardiographic feature is pathognomonic.

Recent Findings

On echocardiography, unusual wall motion abnormalities, which do not fit a classic coronary distribution, along with diastolic dysfunction may alert one to the presence of cardiac sarcoid, particularly in the right clinical context. Myocardial strain imaging on echocardiography may increase the sensitivity of identifying cardiac sarcoidosis. Alternative imaging with cardiac magnetic resonance imaging or positron emission tomography have become more frequently utilized to establish a diagnosis of CS.

Summary

Cardiac sarcoidosis remains a difficult condition to diagnose. However early diagnosis is critical to decrease the associated high mortality. Endomyocardial biopsy is highly specific but lacks sensitivity due to the patchy nature of the granulomatous deposition. Thus, imaging plays a role in diagnosis as well as for follow-up. Echocardiography remains an hallmark during the workup for CS. Decreased sensitivity of echocardiography has facilitated the use of other techniques to establish the presence of CS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

Abbreviations

AV:

Atrioventricular

CMR:

Cardiac magnetic resonance

CS:

Cardiac sarcoidosis

CV:

Cardiovascular

EKG:

Electrocardiogram

EMB:

Endomyocardial biopsy

GLS:

Global longitudinal strain

LV:

Left ventricle/left ventricular

PET/CT:

18F-fleurodeoxyglucose positron emission tomography/computed tomography

PHT:

Pulmonary hypertension

RV:

Right ventricle/right ventricular

TR:

Tricuspid regurgitation

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major Importance

  1. Boeck C. Multiple benign sarcoid of the skin. J Cutan Genitourin Dis. 1899;17:543–50.

    Google Scholar 

  2. Johnson JB, Jason RS. Sarcoidosis of the heart. Am Heart J. 1944;27(2):246–58.

    Article  Google Scholar 

  3. Longcope WT, Freiman DG. A study of sarcoidosis; based on a combined investigation of 160 cases including 30 autopsies from The Johns Hopkins Hospital and Massachusetts General Hospital. Medicine. 1952;31(1):1–132.

    Article  CAS  Google Scholar 

  4. Mirsaeidi M, Machado RF, Schraufnagel D, Sweiss NJ, Baughman RP. Racial difference in sarcoidosis mortality in the United States. Chest. 2015;147(2):438–49.

    Article  Google Scholar 

  5. 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(7):1305–23.

    Article  Google Scholar 

  6. Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet P-Y, Müller-Quernheim J. Sarcoidosis. Lancet. 2014;383(9923):1155–67.

    Article  Google Scholar 

  7. 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(2):167–72.

    CAS  PubMed  Google Scholar 

  8. Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation. 1978;58(6):1204–11.

    Article  CAS  Google Scholar 

  9. Blauwet LA, Cooper LT. Idiopathic giant cell myocarditis and cardiac sarcoidosis. Heart Fail Rev. 2013;18(6):733–46.

    Article  Google Scholar 

  10. Kim JS, Judson MA, Donnino R, Gold M, Cooper LT Jr, Prystowsky EN, et al. Cardiac sarcoidosis. Am Heart J. 2009;157(1):9–21.

    Article  CAS  Google Scholar 

  11. Yigla M, Badarna-Abu-Ria N, Tov N, Ravell-Weiller D, Rubin AH. Sarcoidosis in northern Israel; clinical characteristics of 120 patients. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19(3):220–6.

    PubMed  Google Scholar 

  12. Sharma OP, Maheshwari A, Thaker K. Myocardial sarcoidosis. Chest. 1993;103(1):253–8.

    Article  CAS  Google Scholar 

  13. Zipse MM, Sauer WH. Cardiac sarcoidosis. Curr Cardiol Rep. 2014;16(8):514.

    Article  Google Scholar 

  14. Mantini N, Williams B Jr, Stewart J, Rubinsztain L, Kacharava A. Cardiac sarcoid: a clinician’s review on how to approach the patient with cardiac sarcoid. Clin Cardiol. 2012;35(7):410–5.

    Article  Google Scholar 

  15. Birnie DH, Nery PB, Ha AC, Beanlands RSB. Cardiac sarcoidosis. J Am Coll Cardiol. 2016;68(4):411–21.

    Article  Google Scholar 

  16. •• Murtagh G, Laffin LJ, Beshai JF, Maffessanti F, Bonham CA, Patel AV, et al. Prognosis of myocardial damage in sarcoidosis patients with preserved left ventricular ejection fraction: risk stratification using cardiovascular magnetic resonance. Circ Cardiovasc Imaging. 2016;9(1):e003738 This study highlights the use of cardiac MR to help identify patients with increased cardiovascular morbidity whose echocardiogram is otherwise unremarkable.

    Article  Google Scholar 

  17. Schuller JL, Zipse M, Crawford T, Bogun F, Beshai J, Patel AR, et al. Implantable cardioverter defibrillator therapy in patients with cardiac sarcoidosis. J Cardiovasc Electrophysiol. 2012;23(9):925–9.

    Article  Google Scholar 

  18. Okada DR, Bravo PE, Vita T, Agarwal V, Osborne MT, Taqueti VR, et al. Isolated cardiac sarcoidosis: a focused review of an under-recognized entity. J Nucl Cardiol 2018;25(4):1136–46. https://doi.org/10.1007/s12350-016-0658-1

    Article  Google Scholar 

  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(1):50–63.

    PubMed  PubMed Central  Google Scholar 

  20. Ardehali H, Howard DL, Hariri A, Qasim A, Hare JM, Baughman KL, et al. A positive endomyocardial biopsy result for sarcoid is associated with poor prognosis in patients with initially unexplained cardiomyopathy. Am Heart J. 2005;150(3):459–63.

    Article  Google Scholar 

  21. •• Riemer HJA, Slart AJMG, Patrizio Lancellotti FH, Blankstein R, Schwartz RG, Jaber RR WA, 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. Eur Heart J Cardiovasc Imaging. 2017;18(10):1073–89 An important position paper illustrating the need for multi-modality imaging to detect the presence of cardiac sarcoidosis.

    Article  Google Scholar 

  22. Mehta D, Lubitz SA, Frankel Z, Wisnivesky JP, Einstein AJ, Goldman M, et al. Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing. Chest. 2008;133(6):1426–35.

    Article  Google Scholar 

  23. Youssef G, Beanlands RS, Birnie DH, Nery PB. Cardiac sarcoidosis: applications of imaging in diagnosis and directing treatment. Heart. 2011;97(24):2078–87.

    Article  Google Scholar 

  24. Bargout R, Kelly RF. Sarcoid heart disease: clinical course and treatment. Int J Cardiol. 2004;97(2):173–82.

    Article  Google Scholar 

  25. •• Aggarwal NR, Snipelisky D, Young PM, Gersh BJ, Cooper LT, Chareonthaitawee P. Advances in imaging for diagnosis and management of cardiac sarcoidosis. Eur Heart J Cardiovasc Imaging. 2015;16(9):949–58 Excellent review outlining the approach in the clinical setting to assess for cardiac involvement in sarcoidosis, utilizing multiple diagnostic tools.

    PubMed  Google Scholar 

  26. Focardi M, Picchi A, Nikiforakis N, Bargagli E, Fossi A, Maggiorelli C, et al. Assessment of cardiac involvement in sarcoidosis by echocardiography. Rheumatol Int. 2009;29(9):1051–5.

    Article  Google Scholar 

  27. Smedema J‐P, van Geuns R‐J, Ainslie G, Ector J, Heidbuchel H, Crijns HJGM. Right ventricular involvement in cardiac sarcoidosis demonstrated with cardiac magnetic resonance. ESC Heart Fail. 2017;4(4):535–44.

    Article  Google Scholar 

  28. 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(5):734–41.

    Article  Google Scholar 

  29. Huitema MP, Grutters JC, Rensing BJWM, Reesink HJ, Post MC. Pulmonary hypertension complicating pulmonary sarcoidosis. Neth Hear J. 2016;24(6):390–9.

    Article  CAS  Google Scholar 

  30. Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest. 2010;138(5):1078–85.

    Article  Google Scholar 

  31. Zelcer AA, LeJemtel TH, Jones J, Stahl J. Pericardial tamponade in sarcoidosis. Can J Cardiol. 1987;3(1):12–3.

    CAS  PubMed  Google Scholar 

  32. Darda S, Zughaib ME, Alexander PB, Machado CE, David SW, Saba S. Cardiac sarcoidosis presenting as constrictive pericarditis. Tex Heart Inst J. 2014;41(3):319–23.

    Article  Google Scholar 

  33. Lynch JP, Hwang J, Bradfield J, Fishbein M, Shivkumar K, Tung R. Cardiac involvement in sarcoidosis: evolving concepts in diagnosis and treatment. Semin Respir Crit Care Med. 2014;35(3):372–90.

    Article  Google Scholar 

  34. Zoneraich S, Gupta MP, Mehta J, Zoneraich O, Wessely Z. Myocardial sarcoidosis presenting as acute mitral insufficiency. Chest. 1974;66(4):452–4.

    Article  CAS  Google Scholar 

  35. 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(9):1344–52.

    Article  Google Scholar 

  36. Felekos I, Aggeli C, Gialafos E, Kouranos V, Rapti A, Sfikakis P, et al. Global longitudinal strain and long-term outcomes in asymptomatic extracardiac sarcoid patients with no apparent cardiovascular disease. Echocardiography. 2018;35(6):804–8. https://doi.org/10.1111/echo.13846.

    Article  Google Scholar 

  37. Joyce E, Ninaber MK, Katsanos S, Debonnaire P, Kamperidis V, Bax JJ, et al. Subclinical left ventricular dysfunction by echocardiographic speckle-tracking strain analysis relates to outcome in sarcoidosis. Eur J Heart Fail. 2015;17(1):51–62.

    Article  Google Scholar 

  38. Joyce E, Delgado V, Ninaber MK, Marsan NA. The invisible made visible: multi-modality imaging in the evaluation of cardiac sarcoidosis. Eur Heart J. 2013;34(17):1278.

    Article  Google Scholar 

  39. Joyce E, Kamperidis V, Ninaber MK, Katsanos S, Debonnaire P, Schalij MJ, et al. Prevalence and correlates of early right ventricular dysfunction in sarcoidosis and its association with outcome. J Am Soc Echocardiogr. 2016;29(9):871–8.

    Article  Google Scholar 

  40. Greulich S, Kitterer D, Kurmann R, Henes J, Latus J, Gloekler S, et al. Cardiac involvement in patients with rheumatic disorders: data of the RHEU-M(A)R study. Int J Cardiol. 2016;224:37–49.

    Article  Google Scholar 

  41. • Chareonthaitawee P, Beanlands RS, Chen W, Dorbala S, Miller EJ, Murthy VL, et al. Joint SNMMI-ASNC expert consensus document on the role of 18F-FDG PET/CT in cardiac sarcoid detection and therapy monitoring. J Nucl Med. 2017;58(8):1341–53 Paper focusing on the newest technique for the detection of cardiac sarcoid: PET imaging with a highly specific protocol to detect CS.

    Article  CAS  Google Scholar 

  42. Wicks EC, Menezes LJ, Elliott PM. Improving the diagnostic accuracy for detecting cardiac sarcoidosis. Expert Rev Cardiovasc Ther. 2015;13(2):223–36.

    Article  CAS  Google Scholar 

  43. Costabel U, Skowasch D, Pabst S, Stork S, Tschope C, Allewelt M, et al. Cardiac sarcoidosis: diagnostic and therapeutic algorithms. Pneumologie. 2014;68(2):124–32.

    Article  CAS  Google Scholar 

Download references

Funding

Dr. Kurmann has received funding from the Department of Rheumatology and Clinical Immunology, University of Bern, Bern, Switzerland.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Sunil V. Mankad.

Ethics declarations

Conflict of Interest

Reto Kurmann, Sunil V. Mankad, and Rekha Mankad declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

This article is part of the Topical Collection on Echocardiography

Electronic Supplementary Material

Video 1

(MP4 873 kb)

Video 2

(MP4 714 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kurmann, R., Mankad, S.V. & Mankad, R. Echocardiography in Sarcoidosis. Curr Cardiol Rep 20, 118 (2018). https://doi.org/10.1007/s11886-018-1065-9

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11886-018-1065-9

Keywords

Navigation