Korean Circ J.  2019 Feb;49(2):119-133. 10.4070/kcj.2018.0432.

Management of Arrhythmias Associated with Cardiac Sarcoidosis

• Yada H
• Soejima K

• KMID: 2432130
• DOI: http://doi.org/10.4070/kcj.2018.0432

Abstract

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology. The annual incidence of systemic sarcoidosis is estimated at 10-20 per 100,000 individuals. Owing to the recent advances in imaging modalities, cardiac sarcoidosis (CS) is diagnosed more frequently. The triad of CS includes conduction abnormality, ventricular tachycardia, and heart failure. Atrial and ventricular arrhythmias are caused by either inflammation or scar formation. Inflammation should be treated with immunosuppression and antiarrhythmic agents and scar formation should be treated with antiarrhythmics and/or ablation, in addition to implantable cardioverter defibrillator (ICD) implantation, if necessary. Ablation can provide a good outcome, but it might require bipolar ablation if the critical portion is located mid-myocardium. Late recurrence might be caused by reactivation of sarcoidosis, which would need to be evaluated by positron emission tomography-computed tomography imaging. Risk of sudden cardiac death (SCD) in patients with advanced atrioventricular block is not low, and ICD implantation could be considered instead of a pacemaker. For risk stratification for SCD, late gadolinium enhancement by cardiac magnetic resonance imaging or program stimulation is often used.

Keyword

Arrhythmias, cardiac; Sarcoidosis; Catheter ablation; Immunosuppression; Defibrillators, implantable

MeSH Terms

Arrhythmias, Cardiac*
Atrioventricular Block
Catheter Ablation
Cicatrix
Death, Sudden, Cardiac
Defibrillators
Defibrillators, Implantable
Electrons
Gadolinium
Heart Failure
Humans
Immunosuppression
Incidence
Inflammation
Magnetic Resonance Imaging
Recurrence
Sarcoidosis*
Tachycardia, Ventricular
Gadolinium

Figure

• 

  Figure 1 Management of ventricular arrhythmias in patients with CS. CS = cardiac sarcoidosis; CMR = cardiac magnetic resonance; FDG-PET = 18F-fluorodeoxyglucose-positron emission tomography; F/U = follow-up; Ga = gallium; ICD = implantable cardioverter defibrillator; LGE = late gadolinium enhancement; LVEF = left ventricular ejection fraction; PES = programmed electrical stimulation; RFA = radiofrequency ablation; VT = ventricular tachycardia.

• 

  Figure 2 Management of AVB in patients with CS. AVB = atrioventricular block; CMR = cardiac magnetic resonance; CS = cardiac sarcoidosis; F/U = follow-up; FDG-PET = 18F-fluorodeoxyglucose-positron emission tomography; Ga = gallium; ICD = implantable cardioverter defibrillator; LGE = late gadolinium enhancement; LVEF = left ventricular ejection fraction; PES = programmed electrical stimulation; VT = ventricular tachycardia.

Reference

1. 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; 88:1006–1010.

2. Smedema JP, Snoep G, van Kroonenburgh MP, et al. Cardiac involvement in patients with pulmonary sarcoidosis assessed at two university medical centers in the Netherlands. Chest. 2005; 128:30–35.

3. 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; 128:1629–1637.

4. Birnie DH, Sauer WH, Bogun F, et al. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart Rhythm. 2014; 11:1305–1323.

5. Kandolin R, Lehtonen J, Airaksinen J, et al. Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study. Circulation. 2015; 131:624–632.

6. Patel MR, Cawley PJ, Heitner JF, et al. Detection of myocardial damage in patients with sarcoidosis. Circulation. 2009; 120:1969–1977.

7. Greulich S, Deluigi CC, Gloekler S, et al. CMR imaging predicts death and other adverse events in suspected cardiac sarcoidosis. JACC Cardiovasc Imaging. 2013; 6:501–511.

8. Naruse Y, Sekiguchi Y, Nogami A, et al. Systematic treatment approach to ventricular tachycardia in cardiac sarcoidosis. Circ Arrhythm Electrophysiol. 2014; 7:407–413.

9. Ichinose A, Otani H, Oikawa M, et al. MRI of cardiac sarcoidosis: basal and subepicardial localization of myocardial lesions and their effect on left ventricular function. AJR Am J Roentgenol. 2008; 191:862–869.

10. Cummings KW, Bhalla S, Javidan-Nejad C, Bierhals AJ, Gutierrez FR, Woodard PK. A pattern-based approach to assessment of delayed enhancement in nonischemic cardiomyopathy at MR imaging. Radiographics. 2009; 29:89–103.

11. Okumura W, Iwasaki T, Toyama T, et al. Usefulness of fasting 18F-FDG PET in identification of cardiac sarcoidosis. J Nucl Med. 2004; 45:1989–1998.

12. Ishimaru S, Tsujino I, Takei T, et al. Focal uptake on 18F-fluoro-2-deoxyglucose positron emission tomography images indicates cardiac involvement of sarcoidosis. Eur Heart J. 2005; 26:1538–1543.

13. Vita T, Okada DR, Veillet-Chowdhury M, et al. Complementary value of cardiac magnetic resonance imaging and positron emission tomography/computed tomography in the assessment of cardiac sarcoidosis. Circ Cardiovasc Imaging. 2018; 11:e007030.

14. Crawford T, Mueller G, Sarsam S, et al. Magnetic resonance imaging for identifying patients with cardiac sarcoidosis and preserved or mildly reduced left ventricular function at risk of ventricular arrhythmias. Circ Arrhythm Electrophysiol. 2014; 7:1109–1115.

15. Hulten E, Agarwal V, Cahill M, 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.

16. Coleman GC, Shaw PW, Balfour PC Jr, et al. Prognostic value of myocardial scarring on CMR in patients with cardiac sarcoidosis. JACC Cardiovasc Imaging. 2017; 10:411–420.

17. Murtagh G, Laffin LJ, Beshai JF, 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:e003738.

18. Ahmadian A, Brogan A, Berman J, et al. Quantitative interpretation of FDG PET/CT with myocardial perfusion imaging increases diagnostic information in the evaluation of cardiac sarcoidosis. J Nucl Cardiol. 2014; 21:925–939.

19. Blankstein R, Osborne M, Naya M, et al. Cardiac positron emission tomography enhances prognostic assessments of patients with suspected cardiac sarcoidosis. J Am Coll Cardiol. 2014; 63:329–336.

20. Sperry BW, Tamarappoo BK, Oldan JD, et al. Prognostic impact of extent, severity, and heterogeneity of abnormalities on ¹⁸F-FDG PET scans for suspected cardiac sarcoidosis. JACC Cardiovasc Imaging. 2018; 11:336–345.

21. Wicks EC, Menezes LJ, Barnes A, et al. Diagnostic accuracy and prognostic value of simultaneous hybrid 18F-fluorodeoxyglucose positron emission tomography/magnetic resonance imaging in cardiac sarcoidosis. Eur Heart J Cardiovasc Imaging. 2018. 19:p. 757–767.

22. Soejima K, Yada H. The work-up and management of patients with apparent or subclinical cardiac sarcoidosis: with emphasis on the associated heart rhythm abnormalities. J Cardiovasc Electrophysiol. 2009; 20:578–583.

23. Aizer A, Stern EH, Gomes JA, Teirstein AS, Eckart RE, Mehta D. Usefulness of programmed ventricular stimulation in predicting future arrhythmic events in patients with cardiac sarcoidosis. Am J Cardiol. 2005; 96:276–282.

24. Mehta D, Mori N, Goldbarg SH, Lubitz S, Wisnivesky JP, Teirstein A. Primary prevention of sudden cardiac death in silent cardiac sarcoidosis: role of programmed ventricular stimulation. Circ Arrhythm Electrophysiol. 2011; 4:43–48.

25. Sadek MM, Yung D, Birnie DH, Beanlands RS, Nery PB. Corticosteroid therapy for cardiac sarcoidosis: a systematic review. Can J Cardiol. 2013; 29:1034–1041.

26. Futamatsu H, Suzuki J, Adachi S, et al. Utility of gallium-67 scintigraphy for evaluation of cardiac sarcoidosis with ventricular tachycardia. Int J Cardiovasc Imaging. 2006; 22:443–448.

27. Yodogawa K, Seino Y, Ohara T, Takayama H, Katoh T, Mizuno K. Effect of corticosteroid therapy on ventricular arrhythmias in patients with cardiac sarcoidosis. Ann Noninvasive Electrocardiol. 2011; 16:140–147.

28. Segawa M, Fukuda K, Nakano M, et al. Time course and factors correlating with ventricular tachyarrhythmias after introduction of steroid therapy in cardiac sarcoidosis. Circ Arrhythm Electrophysiol. 2016; 9:e003353.

29. Nagai S, Yokomatsu T, Tanizawa K, et al. Treatment with methotrexate and low-dose corticosteroids in sarcoidosis patients with cardiac lesions. Intern Med. 2014; 53:427–433.

30. Theodore J, Kaur Saggu D, Yalagudri S, Kishore J, Devidutta S, Narasimhan C. Management of refractory ventricular tachycardia due to cardiac sarcoidosis—A biologic approach. HeartRhythm Case Rep. 2018 [Epub ahead of print]..

31. Echt DS, Liebson PR, Mitchell LB, et al. Mortality and morbidity in patients receiving encainide, flecainide, or placebo the cardiac arrhythmia suppression trial. N Engl J Med. 1991; 324:781–788.

32. Kron J, Sauer W, Schuller J, et al. Efficacy and safety of implantable cardiac defibrillators for treatment of ventricular arrhythmias in patients with cardiac sarcoidosis. Europace. 2013; 15:347–354.

33. Betensky BP, Tschabrunn CM, Zado ES, et al. Long-term follow-up of patients with cardiac sarcoidosis and implantable cardioverter-defibrillators. Heart Rhythm. 2012; 9:884–891.

34. Schuller JL, Zipse M, Crawford T, et al. Implantable cardioverter defibrillator therapy in patients with cardiac sarcoidosis. J Cardiovasc Electrophysiol. 2012; 23:925–929.

35. Reek S, Burri H, Roberts PR, et al. The wearable cardioverter-defibrillator: current technology and evolving indications. Europace. 2017; 19:335–345.

36. Papageorgiou N, Providência R, Bronis K, et al. Catheter ablation for ventricular tachycardia in patients with cardiac sarcoidosis: a systematic review. Europace. 2018; 20:682–691.

37. 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; 6:189–195.

38. Dechering DG, Kochhäuser S, Wasmer K, et al. Electrophysiological characteristics of ventricular tachyarrhythmias in cardiac sarcoidosis versus arrhythmogenic right ventricular cardiomyopathy. Heart Rhythm. 2013; 10:158–164.

39. Kumar S, Baldinger SH, Kapur S, et al. Right ventricular scar-related ventricular tachycardia in nonischemic cardiomyopathy: electrophysiological characteristics, mapping, and ablation of underlying heart disease. J Cardiovasc Electrophysiol. 2018; 29:79–89.

40. Muser D, Santangeli P, Castro SA, et al. Long-term outcome after catheter ablation of ventricular tachycardia in patients with nonischemic dilated cardiomyopathy. Circ Arrhythm Electrophysiol. 2016; 9:e004328.

41. Muser D, Santangeli P, Liang JJ, et al. Characterization of the electroanatomic substrate in cardiac sarcoidosis: correlation with imaging findings of scar and inflammation. JACC Clin Electrophysiol. 2018; 4:291–303.

42. Koplan BA, Soejima K, Baughman K, Epstein LM, Stevenson WG. Refractory ventricular tachycardia secondary to cardiac sarcoid: electrophysiologic characteristics, mapping, and ablation. Heart Rhythm. 2006; 3:924–929.

43. Ladyjanskaia GA, Basso C, Hobbelink MG, et al. Sarcoid myocarditis with ventricular tachycardia mimicking ARVD/C. J Cardiovasc Electrophysiol. 2010; 21:94–98.

44. Shiraishi J, Tatsumi T, Shimoo K, et al. Cardiac sarcoidosis mimicking right ventricular dysplasia. Circ J. 2003; 67:169–171.

45. Philips B, Madhavan S, James CA, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear. Circ Arrhythm Electrophysiol. 2014; 7:230–236.

46. Kumar S, Brown G, Sutherland F, et al. The transesophageal echo probe may contribute to esophageal injury after catheter ablation for paroxysmal atrial fibrillation under general anesthesia: a preliminary observation. J Cardiovasc Electrophysiol. 2015; 26:119–126.

47. Sekhri V, Sanal S, Delorenzo LJ, Aronow WS, Maguire GP. Cardiac sarcoidosis: a comprehensive review. Arch Med Sci. 2011; 7:546–554.

48. Nordenswan HK, Lehtonen J, Ekström K, et al. Outcome of cardiac sarcoidosis presenting with high-grade atrioventricular block. Circ Arrhythm Electrophysiol. 2018; 11:e006145.

49. 2012 Writing Group Members, Tracy CM, Epstein AE, et al. 2012 ACCF/AHA/HRS focused update of the 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Thorac Cardiovasc Surg. 2012; 144:e127–45.

50. Cain MA, Metzl MD, Patel AR, et al. Cardiac sarcoidosis detected by late gadolinium enhancement and prevalence of atrial arrhythmias. Am J Cardiol. 2014; 113:1556–1560.

51. Viles-Gonzalez JF, de Castro Miranda R, Scanavacca M, Sosa E, d'Avila A. Acute and chronic effects of epicardial radiofrequency applications delivered on epicardial coronary arteries. Circ Arrhythm Electrophysiol. 2011; 4:526–531.

52. Srivatsa UN, Rogers J. Sarcoidosis and atrial fibrillation: a rare association and interlink with inflammation. Indian Pacing Electrophysiol J. 2012; 12:290–291.

53. Namboodiri N, Stiles MK, Young GD, Sanders P. Electrophysiological features of atrial flutter in cardiac sarcoidosis: a report of two cases. Indian Pacing Electrophysiol J. 2012; 12:284–289.

54. Crawshaw AP, Wotton CJ, Yeates DG, Goldacre MJ, Ho LP. Evidence for association between sarcoidosis and pulmonary embolism from 35-year record linkage study. Thorax. 2011; 66:447–448.

55. Swigris JJ, Olson AL, Huie TJ, et al. Increased risk of pulmonary embolism among US decedents with sarcoidosis from 1988 to 2007. Chest. 2011; 140:1261–1266.

56. Willner JM, Viles-Gonzalez JF, Coffey JO, Morgenthau AS, Mehta D. Catheter ablation of atrial arrhythmias in cardiac sarcoidosis. J Cardiovasc Electrophysiol. 2014; 25:958–963.