. Author manuscript; available in PMC: 2021 Oct 20.

Published in final edited form as: J Am Coll Cardiol. 2020 Oct 20;76(16):1878–1901. doi: 10.1016/j.jacc.2020.08.042

Table 2:

HRS and Japanese Circulation Society Working Group criteria for the diagnosis of CS

HRS criteria for the Diagnosis of CS	Japanese Circulation Society for the Diagnosis of CS (2017)
1. Histological Diagnosis from Myocardial Tissue CS is diagnosed in the presence of non-caseating granuloma on histological examination of myocardial tissue with no alternative cause identified (including negative organismal stains if applicable) 2. Clinical Diagnosis from Invasive and Non-Invasive Studies: It is probable* that there is CS if: a) There is a histological diagnosis of extra-cardiac sarcoidosis and b) One or more of following is present Steroid +/− immunosuppressant responsive cardiomyopathy or heart block Unexplained reduced LVEF (<40%) Unexplained sustained (spontaneous or induced) VT Mobitz type II 2^nd degree heart block or 3^rd degree heart block Patchy uptake on dedicated cardiac PET (in a pattern consistent with CS), Late Gadolinium Enhancement on CMR (in a pattern consistent with CS), Positive gallium uptake (in a pattern consistent with CS) and c) Other causes for the cardiac manifestation(s) have been reasonably excluded *In general, ‘probable involvement’ is considered adequate to establish a clinical diagnosis of CS	1. Histological diagnosis group (those with positive myocardial biopsy findings) 2. Clinical diagnosis group (those without a positive myocardial biopsy) Granulomas are found in organs other than the heart, and clinical findings are strongly suggestive of cardiac involvement; or clinical findings are strongly suggestive of pulmonary or ophthalmic sarcoidosis and at least two of the five characteristic laboratory^# and clinical findings of sarcoidosis are strongly suggestive of CS Clinical findings that strongly suggest the presence of cardiac involvement. 1) Two or more of the five major criteria are satisfied. 2) One of the five major criteria and two or more of the three minor criteria are satisfied. 1. Major criteria (a) High-grade atrioventricular block or fatal ventricular arrhythmia (i.e VT or VF) (b) Basal thinning of the ventricular septum or abnormal ventricular wall anatomy (ventricular aneurysm, thinning of the middle or upper ventricular septum, regional ventricular wall thickening) (c) Left ventricular contractile dysfunction (left ventricular ejection fraction <50%) (d) Abnormalities of ⁶⁷Ga citrate scintigraphy or ¹⁸F-FDG PET (e) Gadolinium-enhanced MRI with delayed contrast enhancement of the heart 2. Minor criteria (f) Abnormal ECG findings: Ventricular arrhythmias (NSVT, multifocal PVCs), bundle branch block, axis deviation, or abnormal Q waves (g) Perfusion defects on myocardial perfusion scintigraphy (SPECT) (h) Endomyocardial biopsy: Monocyte infiltration and myocardial fibrosis #1) Bilateral hilar lymphadenopathy; 2) Elevated ACE, serum lysozyme levels or soluble interleukin-2 receptor (sIL-2R) levels; 3) Abnormalities of ⁶⁷Ga citrate scintigraphy or ¹⁸F-FDG PET; 4) A high percentage of lymphocytes with CD4/CD8 ratio of >3.5 in BAL.

HRS criteria for the Diagnosis of CS

Japanese Circulation Society for the Diagnosis of CS (2017)

1. Histological Diagnosis from Myocardial Tissue
CS is diagnosed in the presence of non-caseating granuloma on histological examination of myocardial tissue with no alternative cause identified (including negative organismal stains if applicable)
2. Clinical Diagnosis from Invasive and Non-Invasive Studies:
It is probable* that there is CS if:
a) There is a histological diagnosis of extra-cardiac sarcoidosis and
b) One or more of following is present

Steroid +/− immunosuppressant responsive cardiomyopathy or heart block
Unexplained reduced LVEF (<40%)
Unexplained sustained (spontaneous or induced) VT
Mobitz type II 2^nd degree heart block or 3^rd degree heart block
Patchy uptake on dedicated cardiac PET (in a pattern consistent with CS), Late Gadolinium Enhancement on CMR (in a pattern consistent with CS), Positive gallium uptake (in a pattern consistent with CS)

and
c) Other causes for the cardiac manifestation(s) have been reasonably excluded
*In general, ‘probable involvement’ is considered adequate to establish a clinical diagnosis of CS

1. Histological diagnosis group (those with positive myocardial biopsy findings)
2. Clinical diagnosis group (those without a positive myocardial biopsy)
Granulomas are found in organs other than the heart, and clinical findings are strongly suggestive of cardiac involvement; or clinical findings are strongly suggestive of pulmonary or ophthalmic sarcoidosis and at least two of the five characteristic laboratory^# and clinical findings of sarcoidosis are strongly suggestive of CS
Clinical findings that strongly suggest the presence of cardiac involvement.
1) Two or more of the five major criteria are satisfied.
2) One of the five major criteria and two or more of the three minor criteria are satisfied.
1. Major criteria
(a) High-grade atrioventricular block or fatal ventricular arrhythmia (i.e VT or VF)
(b) Basal thinning of the ventricular septum or abnormal ventricular wall anatomy (ventricular aneurysm, thinning of the middle or upper ventricular septum, regional ventricular wall thickening)
(c) Left ventricular contractile dysfunction (left ventricular ejection fraction <50%)
(d) Abnormalities of ⁶⁷Ga citrate scintigraphy or ¹⁸F-FDG PET
(e) Gadolinium-enhanced MRI with delayed contrast enhancement of the heart
2. Minor criteria
(f) Abnormal ECG findings: Ventricular arrhythmias (NSVT, multifocal PVCs), bundle branch block, axis deviation, or abnormal Q waves
(g) Perfusion defects on myocardial perfusion scintigraphy (SPECT)
(h) Endomyocardial biopsy: Monocyte infiltration and myocardial fibrosis
#1) Bilateral hilar lymphadenopathy; 2) Elevated ACE, serum lysozyme levels or soluble interleukin-2 receptor (sIL-2R) levels; 3) Abnormalities of ⁶⁷Ga citrate scintigraphy or ¹⁸F-FDG PET; 4) A high percentage of lymphocytes with CD4/CD8 ratio of >3.5 in BAL.

Adapted from (96,136)