Table 1 The Japanese Society of Sarcoidosis and Other Granulomatous Disorders' guidelines for CS diagnosis (2006)

Histological diagnosis group

Cardiac sarcoidosis is confirmed when endomyocardial biopsy specimens demonstrate noncaseating epithelioid cell granulomas with a histological or clinical diagnosis of extracardiac sarcoidosis.

Clinical diagnosis group

Although endomyocardial biopsy specimens do not demonstrate noncaseating epithelioid cell granulomas, extracardiac sarcoidosis is histologically or clinically diagnosed and satisfies the following conditions and more than one in six basic diagnostic criteria.

1. Two or more of the four major criteria are satisfied.

2. One of the four major criteria and two or more of the five minor criteria are satisfied.

Major criteria

a. Advanced atrioventricular block

b. Basal thinning of the interventricular septum

c. Positive 67 gallium uptake in the heart

d. Depressed ejection fraction of the left ventricle (<50%)

Minor criteria

a. Abnormal ECG findings: ventricular arrhythmias (ventricular tachycardia, multifocal or frequent PVCs), CRBBB, axis deviation or abnormal Q-wave

b. Abnormal echocardiography: regional abnormal wall motion or morphological abnormality (ventricular aneurysm, wall thickening)

c. Nuclear medicine: perfusion defect detected by 201 thallium or 99 mtechnetium myocardial scintigraphy

d. Gadolinium-enhanced CMR imaging: delayed enhancement of the myocardium

e. Endomyocardial biopsy: interstitial fibrosis or monocyte infiltration over moderate grade.