Abstract

Cardiac sarcoidosis (CS) is an increasingly recognized cause of heart failure and arrhythmia. Historically challenging to identify, particularly in the absence of extracardiac sarcoidosis, diagnosis of CS has improved with advancements in cardiac imaging. Recognition as well as management may require interpretation of multiple imaging modalities. Echocardiography may serve as an initial screening study for cardiac involvement in patients with systemic sarcoidosis. Cardiac magnetic resonance imaging (CMR) provides information on diagnosis as well as risk stratification, particularly for ventricular arrhythmia in the setting of late gadolinium enhancement. More recently, 18F-fluorodeoxyglucose position emission tomography (FDG-PET) has assumed a valuable role in the diagnosis and longitudinal management of patients with CS, allowing for the assessment of response to treatment. Hybrid FDG-PET/CT may also be used in the evaluation of extracardiac inflammation, permitting the identification of biopsy sites for diagnostic confirmation. Herein we examine the approach to diagnosis and management of CS using multimodality imaging via a case-based review.

Highlights

  • Sarcoidosis is a multiorgan system disease characterized by noncaseating granulomatous inflammation [1,2,3]

  • When there is a histologic diagnosis of extracardiac sarcoidosis, a diagnosis of “probable CS” can be made with the following Heart Rhythm Society (HRS) imaging criteria: reduced left ventricular ejection fraction (LVEF)

  • Persistent LVEF ≤ 35% despite optimal medical therapy and immunosuppression, sustained ventricular tachycardia, and aborted sudden cardiac arrest remain class I indications for an implantable cardioverted-defibrillator (ICD) by the most recent HRS guidelines [9, 54], while class IIa indications include patients with LVEF ≥ 35% and syncope, evidence of myocardial scar by Cardiac magnetic resonance imaging (CMR) or fluorodeoxyglucose position emission tomography (FDG-PET), an indication for permanent pacing, or inducible sustained ventricular arrhythmia on electrophysiological study [54]

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Summary

Introduction

Sarcoidosis is a multiorgan system disease characterized by noncaseating granulomatous inflammation [1,2,3]. The revised Japanese Circulation Society updated criteria for CS to allow for a diagnosis of possible or isolated CS based on imaging characteristics, including cardiac FDG uptake, LGE on CMR, and abnormalities in ventricular wall anatomy and function (basal thinning of the interventricular septum, ventricular aneurysm, LVEF < 50%) [12].

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