Table 1.
There are 2 pathways to a diagnosis of cardiac sarcoidosis (CS): |
1. Histological diagnosis from myocardial tissue |
CS is diagnosed if an endomyocardial biopsy shows non-caseating granuloma with no alternative cause for the histological findings identified |
2. Clinical diagnosis from invasive and non-invasive studies: |
CS is probable* 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 left ventricular ejection fraction (40%) |
➢ Unexplained sustained (spontaneous or induced) ventricular tachycardia |
➢ Mobitz type II, second- or third-degree heart block |
➢ Patchy uptake on dedicated cardiac FDG-PET in a pattern consistent with CS |
➢ Late Gadolinium Enhancement on CMR consistent with CS pattern |
➢ 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.
Adapted from [5] with minor modifications. CMR, cardiac magnetic resonance; FDG-PET, 18F-fluorodeoxyglucose positron emission tomography.