Page 9 - BH-2-4

Brain & Heart Cardiac sarcoidosis with AVB and VA

Table 1. Diagnostic criteria for CS as outlined by JCS, HRS, and WASOG 13‑15
JCS 2016 CS with systemic involvement Isolated CS
Diagnosis confirmed histologically through endomyocardial biopsy Diagnosis confirmed histologically through
OR endomyocardial biopsy
Major criteria* OR
High-degree AVB or fatal VA 67 Ga citrate scintigraphy or 18F-FDG PET revealing
LV contractile dysfunction abnormally high tracer accumulation in the heart
67 Ga citrate scintigraphy or 18F-FDG PET revealing abnormally high AND three of the following
tracer accumulation in the heart High-degree AVB or fatal VA
LGE of the myocardium on CMR LV contractile dysfunction
Basal thinning of ventricular septum or abnormal ventricular wall LGE of the myocardium on CMR
anatomy Basal thinning of the ventricular septum or abnormal
Minor criteria ventricular wall anatomy
Abnormal ECG findings: VA, bundle branch block, axis deviation, AND all of the following
abnormal Q waves No clinical findings of sarcoidosis in organs other than
Perfusion defect on perfusion scintigraphy the heart
Monocyte infiltration and moderate-to-severe myocardial interstitial fibrosis Chest CT showing no hilar lymphadenopathy or shadows
AND along the lymphatic tracts
Granulomas found in other organs in addition to the heart 67 Ga scintigraphy or 18F-FDG PET revealing no
OR abnormal tracer accumulation in organs other than the
Clinical findings suggestive of sarcoidosis in either the respiratory tract heart
or eyes
AND
The presence of two of the following criteria
• Bilateral hilar lymphadenopathy
• Raised serum angiotensin‑converting enzyme activity or lysozyme levels
• High soluble interleukin‑2 receptor levels
• Significant tracer accumulation on Ga citrate scintigraphy or
67
18F-FDG PET
• High percentage of lymphocytes with a CD4/CD8 ratio of >3.5 in
bronchoalveolar lavage fluid
*Where endomyocardial biopsy is negative, either two major or one major
criterion and two or more minor criteria are required to make a diagnosis of CS
HRS 2014 Diagnosis confirmed based on histological finding of non-caseating granulomas on endomyocardial biopsy
Diagnosis of CS is probable if extracardiac sarcoidosis is confirmed and one or more of the following criteria are satisfied:
Immunosuppressant-responsive cardiomyopathy or AVB
Unexplained LVEF <40%
Unexplained sustained VT or high-degree AVB
Patchy FDG uptake on cardiac PET in a pattern consistent with that of CS
LGE on CMR
Positive gallium uptake in a pattern consistent with that of CS
WASOG 2014 Granulomatous inflammation demonstrated in one other organ and one of the following criteria is satisfied:
Treatment-responsive cardiomyopathy or AVB
Reduced LVEF in the absence of other risk factors
Spontaneous or inducible VT with no other risk factors
Mobitz II or complete heart block
Positive gallium uptake in a pattern consistent with that of CS
LGE or T2 prolongation on CMR
Defect on perfusion scintigraphy
Abbreviations: 18F-FDG PET: 18-Fluorodeoxyglucose positron emission tomography; AVB: Atrioventricular block; CMR: Cardiac magnetic resonance
imaging; CS: Cardiac sarcoidosis; CT: Computerized tomography; ECG: Electrocardiogram; HRS: Heart Rhythm Society; JCS: Japanese Circulation
Society; LGE: Late gadolinium enhancement; LV: Left ventricle; LVEF: Left ventricular ejection fraction; VA: Ventricular arrhythmia; VT: Ventricular
tachycardia; WASOG: World Association on Sarcoidosis and Other Granulomatous Disorders.

Furthermore, a reduced left ventricular ejection fraction basal septum is a major diagnostic criterion according to
(LVEF) is a significant independent risk factor for the JCS guidelines. 13
mortality. Detection of echocardiographic findings at the A study by Di Stefano et al. explored the use of left
21
22
initial presentation with AVB and VAs is associated with and right ventricular global longitudinal strain assessed
earlier diagnosis and treatment of CS. 10,13 Thinning of the through speckle-tracking echocardiography. They found a

Volume 2 Issue 4 (2024) 3 doi: 10.36922/bh.3515