Imaging of Cardiac Sarcoidosis: An Update and Future Aspects

Sarcoidosis is a multisystem inflammatory disease characterized histologically by the presence of noncaseating granulomas. Sarcoidosis primarily affects the pulmonary and lymphatic systems, but can involve nearly any organ system.1 Although the exact etiology of sarcoidosis is unclear, it is thought to be multifactorial, involving the convergence of environmental and genetic risk factors that ultimately result in an exaggerated inflammatory and immune response to an unknown antigenic trigger2. The exact prevalence of sarcoidosis is not known, ranging from 100 to 330 per 100,000 individuals in the United States (US), and appears to be influenced by multiple factors including sex, race, and geographic location.3, 4, 5 Cardiac involvement (CS) can occur in up to 75% of patients with sarcoidosis, with an estimated annual incidence of 10 to 40 per 100,000 individuals in the US and Europe, and can account for 25% of sarcoid-related mortality.6, 7, 8, 9 Although frequently initially asymptomatic, the presentation of CS can include conduction disturbances (most common), arrhythmias, heart failure, and in up to 35% of patients, sudden cardiac death, which can sometimes represent the index presentation.6,7,10, 11, 12, 13, 14 The overall survival of patients with CS is estimated to be between 60% and 90% at 5 years, with ventricular arrhythmia being the most common cause of mortality.11,15 Thus, early diagnosis of this condition is imperative to ensure prompt initiation of treatment and appropriate subsequent monitoring. Screening for CS has been advocated by the 2014 Heart Rhythm Consensus Statement in individuals with extracardiac sarcoidosis, and should be performed with a thorough cardiac history (Class I), a 12-lead electrocardiogram (Class I), and a resting transthoracic echocardiogram (Class IIa).16

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