Cardiac Sarcoidosis: A Review of Contemporary Challenges in Diagnosis and Treatment

Abstract

Sarcoidosis is a systemic disease characterized by noncaseating granulomas and is often a diagnosis of exclusion. The actual prevalence of cardiac sarcoidosis (CS) is unknown, as studies have demonstrated mixed data. CS may be asymptomatic and is likely more frequently encountered than previously thought. Sudden death may often be the presenting feature of CS. Most deaths attributed to CS are caused by arrhythmias or conduction system disease, and congestive heart failure may occur. Current expert consensus on diagnosis of CS continues to rely on endomyocardial biopsy, in the absence of which, histologic proof of extracardiac sarcoid involvement is necessitated. Emergence of newer noninvasive imaging modalities such as cardiac magnetic resonance imaging and positron emission tomography, have become increasingly popular tools utilized in patients with both clinical and asymptomatic CS, and have demonstrated good diagnostic capability. The main therapeutic approaches in patients with CS can be broadly divided into the following 2 categories: pharmacological management and invasive or device oriented. However, much remains unknown about the optimal screening protocols of asymptomatic patients with extracardiac sarcoidosis and treatment of biopsy-proven CS. Our knowledge about CS has amplified significantly over the last 30 years and the growing realization that this process is often asymptomatic is paving the way for better screening protocols and earlier detection of this serious condition.

Keywords: Cardiac sarcoidosis; Endomyocardial biopsy; Immunosuppressive therapy; Late gadolinium enhancement; Noninvasive imaging.