Arrhythmic manifestations and outcomes of definite and probable cardiac sarcoidosis

• Published in: Heart rhythm Vol. 21; no. 10; pp. 1978 - 1986
• Main Authors: Sykora, Daniel, Rosenbaum, Andrew N., Churchill, Robert A., Kim, B. Michelle, Elwazir, Mohamed Y., Bois, John P., Giudicessi, John R., Bratcher, Melanie, Young, Kathleen A., Ryan, Sami M., Sugrue, Alan M., Killu, Ammar M., Chareonthaitawee, Panithaya, Kapa, Suraj, Deshmukh, Abhishek J., Abou Ezzeddine, Omar F., Cooper, Leslie T., Siontis, Konstantinos C.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.10.2024
• Subjects: Cardiac sarcoidosis; Cardiomyopathy; Cardiovascular implantable electronic device; Sudden cardiac death; Ventricular arrhythmia; Cardiovascular implantable electronic device; VT/VF; Cardiomyopathy; CI; Ventricular arrhythmia; CMR; HR; 18F-FDG; ICD; IQR; Sudden cardiac death; Cardiac sarcoidosis; CS; CIED; PET
• ISSN: 1547-5271; 1556-3871; 1556-3871
• DOI: 10.1016/j.hrthm.2024.04.009

The 2014 Heart Rhythm Society consensus statement defines histological (definite) and clinical (probable) diagnostic categories of cardiac sarcoidosis (CS), but few studies have compared their arrhythmic phenotypes and outcomes. The purpose of this study was to evaluate the electrophysiological/arrhythmic phenotype and outcomes of patients with definite and probable CS. We analyzed the arrhythmic/electrophysiological phenotype in a single-center North American cohort of 388 patients (median age 56 years; 39% female, n = 151) 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) event or sudden cardiac death. Key secondary outcomes were also assessed. At index evaluation, in situ cardiac implantable electronic devices and antiarrhythmic drug use were more common in definite CS. At a median follow-up of 3.1 years, the primary outcome occurred in 22 patients with definite CS (38%) and 127 patients with probable CS (38%) (log-rank, P = .55). In multivariable analysis, only a higher ratio of the 18F-fluorodeoxyglucose maximum standardized uptake value of the myocardium to the maximum standardized uptake value of the blood pool (hazard ratio 1.09; 95% confidence interval 1.03–1.15; P = .003, per 1 unit increase) was associated with the primary outcome. During follow-up, patients with definite CS had a higher burden of device-treated VT/VF events (mean 2.86 events per patient-year vs 1.56 events per patient-year) and a higher rate of progression to heart transplant/left ventricular assist device implantation but no difference in all-cause mortality compared with patients with probable CS. Patients with definite and probable CS had similarly high risks of first sustained VT/VF/sudden cardiac death and all-cause mortality, though patients with definite CS had a higher overall arrhythmia burden. Both CS diagnostic groups as defined by the 2014 Heart Rhythm Society criteria require an aggressive approach to prevent arrhythmic complications.