Ten Questions Cardiologists Should Be Able to Answer About Cardiac Sarcoidosis: Case-Based Approach and Contemporary Review

Abstract

Sarcoidosis is an inflammatory multisystemic disease of unknown etiology characterized by the formation of noncaseating epithelioid cell granulomas. Cardiac sarcoidosis might be life-threatening and its diagnosis and treatment remain a challenge nowadays. The aim of this review is to provide an updated overview of cardiac sarcoidosis and, through 10 practical clinical questions and real-life challenging case scenarios, summarize the main clinical presentation, diagnostic criteria, imaging findings, and contemporary treatment.

Heart block in a 60-year-old man with undiagnosed cardiac sarcoidosis

“… 9 Cardiac involvement in sarcoidosis has been reported in as many as 27% of autopsy studies in some North American cohorts, but many cases may be undiagnosed. 10 …”

What cardiologists should know about cardiac sarcoidosis in 2022?

“…CMR is better at detecting the chronic fibrotic stage of CS and has a good prognostic role with late gadolinium enhancement (LGE) quantification usually present in the subepicardium and midmyocardium [18]. Indeed, multiple studies have shown an association between LGE and increased mortality and ventricular arrhythmias [1 ▪ ,19]. On the other hand, PET/CT performs better at detecting the active inflammatory stage of CS and is a good tool for the follow-up after initiation of IT [1 ▪ ].…”

“…Indeed, multiple studies have shown an association between LGE and increased mortality and ventricular arrhythmias [1 ▪ ,19]. On the other hand, PET/CT performs better at detecting the active inflammatory stage of CS and is a good tool for the follow-up after initiation of IT [1 ▪ ].…”

“…Regardless of the scarcity of data, clinician and experts mostly agree that symptomatic patients with inflammation on cardiac FDG-PET imaging should be treated with IT including prednisone as first-line treatment [30 ▪ ,35,36,39 ▪ ,40,41]. Indeed, the mode of presentation of CS is frequently catastrophic and often preclude the clinician from not starting IT [1 ▪ ]. A retrospective study by Yazaki et al demonstrated that starting corticosteroids before the occurrence of systolic dysfunction resulted in excellent clinical outcomes [42].…”

“…Nearby sixty percentage of patients diagnosed with cardiac sarcoidosis (CS) are not known for an extra-CS at the time of their initial presentation but 90% will show evidence of extracardiac disease after thorough investigations [1 ▪ ,2,3]. These patients have a clinically isolated CS [1 ▪ ] and typically present high-degree atrioventricular block (AVB), ventricular tachycardia (VT), heart failure (HF) or sudden cardiac death (SCD). It is therefore recommended by most expert consensus to investigate for CS in young patients presenting one of these severe clinical manifestations [1 ▪ ,4].…”