Evaluation of Known or Suspected Cardiac Sarcoidosis

Abstract

Abstract-Sarcoidosis is a multisystem disorder of unknown cause, and cardiac sarcoidosis affects at least 25% of patients and accounts for substantial mortality and morbidity from this disease. Cardiac sarcoidosis may present with heart failure, left ventricular systolic dysfunction, AV block, atrial or ventricular arrhythmias, and sudden cardiac death. Cardiac involvement can be challenging to detect and diagnose because of the focal nature of the disease, as well as the fact that clinical criteria have limited diagnostic accuracy. Nevertheless, the diagnosis of cardiac sarcoidosis can be enhanced by integrating both clinical and imaging findings. This article reviews the various roles that different imaging modalities provide in the evaluation and management of patients with known or suspected cardiac sarcoidosis. (Circ Cardiovasc Imaging. 2016;9:e000867.

Joint SNMMI–ASNC expert consensus document on the role of 18F-FDG PET/CT in cardiac sarcoid detection and therapy monitoring

“…30,35,37,[46][47][48][49][50] In the absence of tissue confirmation of CS, there is agreement that the diagnosis of cardiac involvement requires integrating multiple sources of data, including 18 F-FDG PET in some cases. Consequently, the diagnosis of CS should not be based on 18 F-FDG PET alone.…”

“…47,68 A normal PET examination for CS will show complete suppression of 18 F-FDG from the myocardium and normal resting myocardial perfusion ( Figure 3, first column). Incomplete suppression of 18 F-FDG from normal myocardium, as might occur because of inadequate patient preparation, may be accompanied by a pattern of diffuse homogeneous 18 F-FDG uptake (Figure 3, second column).…”

“…In the presence of active inflammation, focal areas of 18 F-FDG uptake will be present without (Figure 3, third column) or with ( Figure 3, fourth and fifth columns) perfusion defects. Although some have described 18 F-FDG uptake without a resting perfusion defect as representing early CS, there 47 is no agreement or evidence that CS progresses in a linear fashion, and not all patients who have inflammation develop scarring.…”

“…Bottom patient with marked response of both extraCS and CS to treatment. Reprinted with permission from Blankstein et al 47 survival rate of 75% and a 10-y survival rate of 61%. 23 Survival curves did not significantly differ between patients treated with an initial prednisone dose of more than 30 mg daily and those treated with 30 mg or less.…”

Multimodality imaging in the diagnosis and management of cardiac sarcoidosis

“…9 Being an extracellular contrast agent, Gadolinium has a slower washout period from areas of scar, a property that allows it to highlight even the smallest areas of fibrosis. 10 As formation of noncaseating granulomas takes place, there is an increase in the amount of LGE as a result of extracellular space expansion and scar formation. 10 When compared to the gold standard (JMHWG), Smedema and colleagues reported CMR with LGE to be 100% sensitive for CS, making it the preferred initial imaging modality for patients with extracardiac sarcoid undergoing a workup for suspected CS.…”