Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Study
  • Published:

Cardiac sarcoidosis concealed by arrhythmogenic right ventricular dysplasia/cardiomyopathy

Nature Clinical Practice Cardiovascular Medicine volume 5, pages 231–236 (2008)Cite this article

Abstract

Background A 37-year-old male with a history of palpitations and ventricular tachycardia was diagnosed with arrhythmogenic right ventricular dysplasia/cardiomyopathy on the basis of clinical assessment, electrocardiography and echocardiography. Over the following 3 years the patient progressed to end-stage heart failure and eventually underwent heart transplantation. Histological analysis of the explanted heart revealed the presence of numerous noncaseating granulomas.

Investigations Electrocardiography, echocardiography, 24 h Holter monitoring, cardiac MRI, coronary angiography, endomyocardial biopsy, exercise testing, electrophysiological study, laboratory examinations and histological examination of the explanted heart.

Diagnosis Cardiac sarcoidosis.

Management Immunosupressive and corticosteroid therapy. Routine endomyocardial biopsy is planned.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Freeze frames from a functional MRI study performed on the patient at presentation.
Figure 2: Typical electrocardiograms recorded from the patient 3 years after his initial presentation.
Figure 3: Echocardiograms recorded from the patient 4 years and 9 months after his initial presentation—8 months before he underwent cardiac transplantation.
Figure 4: Examination of the explanted heart revealed findings that were typical of cardiac sarcoidosis.

References

  1. Thomas KW and Hunninghake GW (2003) Sarcoidosis. JAMA 289: 3300–3303

    Article  Google Scholar 

  2. Chapelon-Abric C et al. (2004) Cardiac sarcoidosis: a restrospective study of 41 cases. Medicine (Baltimore) 83: 315–334

    Article  Google Scholar 

  3. Sekiguchi M et al. (1980) Clinical and histological profile of sarcoidosis of the heart and acute idiopathic myocarditis: concepts through a study employing myocardial biopsy I—sarcoidosis. Jpn Circ J 44: 249–263

    Article  CAS  Google Scholar 

  4. Shimada T et al. (2001) Diagnosis of cardiac sarcoidosis and evaluation of the effects of steroid therapy by Gadolinium-DTPA-enhanced magnetic resonance imaging. Am J Med 110: 520–527

    Article  CAS  Google Scholar 

  5. Ardehali H et al. (2005) A positive endomyocardial biopsy result for sarcoid is associated with poor prognosis in patients with initially unexplained cardiomyopathy. Am Heart J 150: 459–463

    Article  Google Scholar 

  6. Marcus FI and Fontaine G (1995) Arrhythmogenic right ventricular dysplasia/cardiomyopathy: a review. Pacing Clin Electrophysiol 18: 1298–1314

    Article  CAS  Google Scholar 

  7. McKenna WJ et al. (1994) Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. Br Heart J 71: 215–218

    Article  CAS  Google Scholar 

  8. Ott P et al. (2003) Cardiac sarcoidosis masquerading as right ventricular dysplasia. Pacing Clin Electrophysiol 26: 1498–1503

    Article  Google Scholar 

  9. Takada K et al. (1994) Prognosis after pacemaker implantation in cardiac sarcoidosis in Japan: clinical evaluation of corticosteroid therapy. Sarcoidosis 11: 113–117

    CAS  PubMed  Google Scholar 

  10. Baughman RP et al. (2003) Sarcoidosis. Lancet 361: 1111–1118

    Article  CAS  Google Scholar 

  11. Winters SL et al. (1991) 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 18: 937–943

    Article  CAS  Google Scholar 

  12. Aizer A et al. (2005) Usefulness of programmed ventricular stimulation in predicting future arrhythmic events in patients with cardiac sarcoidosis. Am J Cardiol 96: 276–282

    Article  Google Scholar 

  13. Burke WM et al. (1990) Transmission of sarcoidosis via cardiac transplantation. Lancet 336: 1579

    Article  CAS  Google Scholar 

  14. Yager JE et al. (2005) Recurrence of cardiac sarcoidosis in a heart transplant recipient. J Heart Lung Transplant 24: 1988–1990

    Article  Google Scholar 

  15. Oni A et al. (1992) Recurrence of sarcoidosis in a cardiac allograft: control with augmented corticosteroids. J Heart Lung Transplant 11: 367–369

    CAS  PubMed  Google Scholar 

Download references

Acknowledgements

P Petrakopoulou and M Greif contributed equally to the preparation of this article.

Author information

Authors and Affiliations

  1. M Greif is a Senior House Officer and C Reithmann and M Nabauer are Consultants in the Department of Cardiology, M Weiss is a Consultant in the Department of Pathology, B Reichart is a Professor of Cardiac Surgery and Chairman of the Department of Cardiac Surgery and G Steinbeck is Professor of Internal Medicine and Cardiology and Chairman of the Department of Cardiology at the University Hospital of Munich, Munich, Germany.,

    Martin Greif, Max Weiss, Christopher Reithmann, Bruno Reichart, Michael Nabauer & Gerhard Steinbeck

  2. P Petrakopoulou is a Senior House Officer in the Department of Cardiology at St Luke's Hospital, Thessaloniki, Greece.,

    Paraskevi Petrakopoulou

Authors
  1. Martin Greif
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Paraskevi Petrakopoulou
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Max Weiss
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Christopher Reithmann
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Bruno Reichart
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Michael Nabauer
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Gerhard Steinbeck
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Paraskevi Petrakopoulou.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Greif, M., Petrakopoulou, P., Weiss, M. et al. Cardiac sarcoidosis concealed by arrhythmogenic right ventricular dysplasia/cardiomyopathy. Nat Rev Cardiol 5, 231–236 (2008). https://doi.org/10.1038/ncpcardio1146

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncpcardio1146

This article is cited by

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing