Korean Circ J.  2012 Aug;42(8):571-574. 10.4070/kcj.2012.42.8.571.

Cardiac Sarcoidosis Presenting With Complete Atrioventricular Block and Sustained Monomorphic Ventricular Tachycardia

  • 1Cardiovascular Center and Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
  • 2Division of Cardiology, Department of Internal Medicine, College of Medicine, Seoul National University and Cardiovascular Center, Seoul National University Bundang Hospital, Seongnam, Korea. djchoi@snu.ac.kr


Sarcoidosis is a rare but potentially fatal multisystem granulomatous disease of unknown etiology. While a number of clinical manifestations may develop, cardiac involvement (prior to or coincident with sarcoidosis of other organs) is an important prognostic factor. Recently, we encountered a patient with cardiac sarcoidosis who presented with complete atrioventricular (AV) block and sustained ventricular tachycardia. An implantable cardioverter-defibrillator was inserted as a precautionary measure for ventricular tachycardia and symptomatic complete AV block. 18F-fluoro-2-deoxyglucose positron emission tomography confirmed a dramatic response to high-dose steroid at four weeks, as demonstrated by a marked decrease in cardiac sarcoid activity from baseline status.


Sarcoidosis; Heart failure; Magentic resonance imaging; Tachycardia, ventricular

MeSH Terms

Atrioventricular Block
Defibrillators, Implantable
Heart Failure
Positron-Emission Tomography
Tachycardia, Ventricular


  • Fig. 1 Initial presentation of the patients. A: admission electrocardiogram: complete atrioventricular block (heart rate, 27 beats per minute). B: echocardiogram: RV and right atrium enlargement and severe RV dysfunction, without pulmonary hypertension (pulmonary artery systolic pressure, 27.64 mm Hg). C: chest X-ray: mild cardiomegaly (cardiothoracic ratio 55%); no hilar lymph node enlargement. D: Gadolinium-dietylene triamine penta-acetic acid-enhanced cardiac MRI: delayed hyper-enhancement of the entire RV wall and subepicardial (apical) or transmural (apical, basal) portions of the left ventricle. E: sustained ventricular tachycardia during endomyocardial biopsy. RV: right ventricle.

  • Fig. 2 Pathologic finding of lymph nodes and results of initial and follow-up 18F-FDG-PET. A and B: right paratracheal (17.27×8.35 mm) and subcarinal (18.63×11.97 mm) lymph node enlargement discovered incidentally in coronary CT angiography six months prior. C and D: lymph node aspirate: noncaseating epitheloid granulomata with giant cells. E and F: baseline 18F-FDG-PET: inhomogeneous uptake, accentuated in left ventricular myocardium {maximum standardized uptake value (SUVMAX)=7.9 g/mL}. G and H: 18F-FDG-PET at one-month follow-up; marked decrease in myocardial hypermetabolism (SUVMAX=3.1 g/mL). 18F-FDG-PET: 18F-fluoro-2-deoxyglucose positron emission tomography.


1. Hagemann GJ WK. Jones Williams W, Davies BH, editors. The clinical, electrocardiographic and pathological features of cardiac sarcoid. Sarcoidosis and Other Granulomatous Disorders: Proceedings of the 8th International Conference Cardiff. 1980. Cardiff: Alpha & Omega Press;601–606.
2. Johns CJ, Michele TM. The clinical management of sarcoidosis: a 50-year experience at the Johns Hopkins Hospital. Medicine (Baltimore). 1999. 78:65–111.
3. 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.
4. Hiraga H, Yuwai K, Hiroe M. Guidelines for the Diagnosis of Cardiac Sarcoidosis: Study Report of Diffuse Pulmonary Diseases. 1993. Tokyo: Japanese Ministry of Health and Welfare;23–24.
5. Chapelon-Abric C, de Zuttere D, Duhaut P, et al. Cardiac sarcoidosis: a retrospective study of 41 cases. Medicine (Baltimore). 2004. 83:315–334.
6. Hillerdal G, Nöu E, Osterman K, Schmekel B. Sarcoidosis: epidemiology and prognosis: a 15-year European study. Am Rev Respir Dis. 1984. 130:29–32.
7. 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. 145:234–241.
8. Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation. 1978. 58:1204–1211.
9. Matsui Y, Iwai K, Tachibana T, et al. Clinicopathological study of fatal myocardial sarcoidosis. Ann N Y Acad Sci. 1976. 278:455–469.
10. 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. 18:937–943.
11. 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. 110:520–527.
12. 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. 122:1895–1901.
13. Uemura A, Morimoto S, Hiramitsu S, Kato Y, Ito T, Hishida H. Histologic diagnostic rate of cardiac sarcoidosis: evaluation of endomyocardial biopsies. Am Heart J. 1999. 138(2 Pt 1):299–302.
14. Ohira H, Tsujino I, Yoshinaga K. 18F-Fluoro-2-deoxyglucose positron emission tomography in cardiac sarcoidosis. Eur J Nucl Med Mol Imaging. 2011. 38:1773–1783.
15. Braun JJ, Kessler R, Constantinesco A, Imperiale A. 18F-FDG PET/CT in sarcoidosis management: review and report of 20 cases. Eur J Nucl Med Mol Imaging. 2008. 35:1537–1543.
16. Kim JS, Judson MA, Donnino R, et al. Cardiac sarcoidosis. Am Heart J. 2009. 157:9–21.
17. Reuhl J, Schneider M, Sievert H, Lutz FU, Zieger G. Myocardial sarcoidosis as a rare cause of sudden cardiac death. Forensic Sci Int. 1997. 89:145–153.
18. Takada K, Ina Y, Yamamoto M, Satoh T, Morishita M. Prognosis after pacemaker implantation in cardiac sarcoidosis in Japan: clinical evaluation of corticosteroid therapy. Sarcoidosis. 1994. 11:113–117.
19. Belhassen B, Pines A, Laniado S. Failure of corticosteroid therapy to prevent induction of ventricular tachycardia in sarcoidosis. Chest. 1989. 95:918–920.
Full Text Links
  • KCJ
export Copy
  • Twitter
  • Facebook
Similar articles
Copyright © 2023 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr