Multimodality evaluation of cardiac sarcoidosis

Excerpt

Cardiac involvement is less common but a main cause of death for patients with sarcoidosis. An accurate and noninvasive diagnosis during the early phase is desirable because treatment with corticosteroids improves the prognosis in some patients. Criteria for diagnosis have been changed and noninvasive imaging has become more important.1 We report a case of cardiac sarcoidosis with multimodality imaging. A 34-year-old man presented with respiratory discomfort and wheezing. He had a first-degree atrioventricular block on electrocardiography and cardiac enlargement with dysfunction on echocardiography. There was no coronary stenosis on coronary angiography. ^99mTc-MIBI scintigraphy revealed broad and severe reduction in myocardial blood flow except for the apical wall and part of the anterior wall (Figure 1). On MRI, late gadolinium enhancement (LGE) was detected in the epicardial and mid layers within the area, consistent with reduced perfusion (Figure 2).2 ¹⁸F-FDG PET/CT revealed focally abnormal uptake in the not only myocardium but also lung, liver, spleen, lymph nodes, and bones, consistent with sarcoidosis (Figure 3).3 The diagnosis of sarcoidosis was pathologically confirmed in a skin biopsy by the presence of typical noncaseating granuloma surrounded by multinucleated giant cells, and the patient was diagnosed as cardiac sarcoidosis according to the revised guidelines of the Japanese Society of Sarcoidosis (2006) (Figure 4). After 4 weeks of steroid therapy, abnormal ¹⁸F-FDG uptakes were reduced, indicating the attenuation of inflammation whereas there were no changes in cardiac hypoperfusion shown by ^99mTc-MIBI scintigraphy (Figure 5).

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