Arrhythmic Manifestations and Outcomes of Definite and Probable Cardiac Sarcoidosis.

BACKGROUND: The 2014 HRS consensus statement defines histological (definite) and clinical (probable) diagnostic categories of cardiac sarcoidosis (CS), but few studies have compared their arrhythmic phenotypes and outcomes.

OBJECTIVE: Evaluate the electrophysiologic/arrhythmic phenotype and outcomes of patients with definite and probable CS.

METHODS: We analyzed the arrhythmic/electrophysiologic phenotype in a single-center North American cohort of 388 patients (median age 56 years, 39% female) diagnosed with definite (n=58) or probable (n=330) CS (2000-2022). The primary composite outcome was survival to first ventricular tachycardia/fibrillation (VT/VF) or sudden cardiac death (SCD). Key secondary outcomes were also assessed.

RESULTS: At index evaluation, in situ cardiac implantable electronic devices and antiarrhythmic drug use were more common in definite CS. At median follow-up 3.1 years, the primary outcome occurred in 22 (38%) definite and 127 (38%) probable CS patients (log-rank p=0.55). In multivariable analysis, only higher 18F-FDG SUVmax myocardium:SUVmax blood pool ratio (HR 1.09 [95% CI 1.03, 1.15], p=0.003, per 1 unit increase) was associated with the primary outcome. During follow-up, definite CS patients had higher burden of device-treated VT/VF events (mean 2.86 vs 1.56 per patient-year) and higher rate of progression to heart transplant/left ventricular assist device implantation, but no difference in all-cause mortality compared to probable CS patients.

CONCLUSION: Definite and probable CS patients had similarly high risks of first sustained VT/VF/SCD and all-cause death though definite CS patients had a higher overall arrhythmic burden. Both CS diagnostic groups as defined by the 2014 HRS criteria require an aggressive approach to prevent arrhythmic complications.