Abstract

The diagnosis of cardiac sarcoidosis (CS) is often suspected based on clinical features when histologic evidence is lacking. While the arrhythmic characteristics of CS with histologic evidence meeting the 2014 Heart Rhythm Society criteria (definite or probable CS) have been extensively studied, the arrhythmic presentations and outcomes of clinical CS are less known. To compare arrhythmia manifestations, management, and outcomes between clinically diagnosed and definite/probable CS. We retrospectively analyzed a single-center referral cohort of patients with available electrophysiologic data stratified by CS category (definite/probable or clinical). Patients with clinical CS had no histologic evidence of cardiac or extracardiac sarcoidosis (biopsy negative or not performed) and the diagnosis was based on clinical course (unexplained sustained VT, Mobitz II, or complete AV block) and imaging features (patchy uptake on dedicated cardiac positron emission tomography or late gadolinium enhancement on cardiac magnetic resonance imaging). History of arrhythmias at index evaluation, arrhythmia management, and outcomes were comparatively assessed for patients with definite/probable CS compared to those with clinical CS. The cohort included 629 CS patients (median age 57 years, male 67%, NYHA class I-II 78%), including 12% definite, 54% probable, and 34% clinical CS patients. History of ventricular and supraventricular arrhythmias was more common in clinical CS compared to definite/probable CS (Figure). Accordingly, the rate of antiarrhythmic drug use was higher in clinical (33%) than definite/probable CS (22%, p=0.002). Median follow-up after index evaluation at our institution was 3.1y (IQR 4.2y). The number of incident VT/VF events per patient per year was greater in clinical CS than definite/probable CS (Figure). Catheter ablations for ventricular arrhythmia were more frequent in clinical CS (27%) than definite/probable CS (9%, p<0.001). Patients with clinical CS exhibited a more severe arrhythmic phenotype compared to patients with definite/probable CS. These findings have implications for the management and prognosis of CS patients within this common clinical category.

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