Table 2.

Comparison of Current Diagnostic Criteria for Cardiac Sarcoidosis

Japanese Ministry of Health and Welfare (2006)Heart Rhythm Society (2014)
Histological diagnosis (definite CS)EMB: noncaseating granulomas and Histological or clinical diagnosis of extra-cardiac sarcoidosisEMB: noncaseating granulomas AND No alternative cause identified
Clinical diagnosisExtra-cardiac sarcoidosis per histological or clinical criteria plus
 ≥2 major clinical criteria for CS or
 1 major plus ≥2 minor criteria for CS
Probable CS
 Extra-cardiac sarcoidosis per histological criteria and
 ≥1 clinical criteria and Alternative causes reasonably excluded
Clinical criteriaMajor
 Advanced AV block Mobitz type II second-degree heart block or third-degree heart block
 Thinning of basal IV septum Positive gallium-67 uptake in heart
 Positive gallium-67 uptake in heart Unexplained LVEF <40%
 LVEF <50% ECG: unexplained sustained (spontaneous or induced) VT
Minor CMR: LGE in a pattern consistent with CS
 ECG: VT, multifocal or frequent PVCs, complete RBBB, axis deviation or Q waves FDG-PET: Patchy uptake in a pattern consistent with CS
 Echo: RWMA, aneurysm, wall thickening Cardiomyopathy or heart block responsive to corticosteroid therapy
 Nuclear: perfusion defects on thallium-201 or technetium-99 m SPECT
 CMR: LGE
 EMB: >moderate interstitial fibrosis or monocyte infiltration
  • CMR indicates cardiac magnetic resonance imaging; CS, cardiac sarcoidosis; EMB, endomyocardial biopsy; FDG-PET, fluorodeoxyglucose- positron emission tomography; LGE, late gadolinium enhancement; LVEF, left ventricular ejection fraction; PVC, premature ventricular contraction; RBBB, right bundle branch block; RWMA, regional wall motion abnormality; SPECT, single-photon emission computed tomography; and VT, ventricular tachycardia.