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Arrhythmic manifestations and outcomes of definite and probable cardiac sarcoidosis
Daniel Sykora1, Andrew N Rosenbaum1, Robert A Churchill2
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
Insights
Patients with definite and probable cardiac sarcoidosis (CS) face similar risks of sudden cardiac death and mortality. However, definite CS patients experience a higher burden of arrhythmias, necessitating aggressive management for both groups.
Area of Science:
- Cardiology
- Electrophysiology
- Sarcoidosis Research
Background:
- The 2014 Heart Rhythm Society (HRS) consensus statement established diagnostic criteria for cardiac sarcoidosis (CS), categorizing it as definite (histological) or probable (clinical).
- Limited research has compared the distinct arrhythmic profiles and clinical outcomes between these two diagnostic categories of CS.
Purpose of the Study:
- To investigate and compare the electrophysiological and arrhythmic characteristics, as well as clinical outcomes, in patients diagnosed with definite versus probable cardiac sarcoidosis.
Main Methods:
- A single-center North American cohort of 388 patients diagnosed with definite (n=58) or probable (n=330) CS between 2000 and 2022 was analyzed.
- The study evaluated the arrhythmic/electrophysiological phenotype, with the primary composite outcome being survival to the first event of ventricular tachycardia/fibrillation (VT/VF) or sudden cardiac death.
Main Results:
- While the primary outcome (first VT/VF or sudden cardiac death) occurred in 38% of both definite and probable CS patients over a median follow-up of 3.1 years, definite CS patients showed a higher burden of device-treated VT/VF events.
- A higher ratio of myocardial to blood pool 18F-fluorodeoxyglucose maximum standardized uptake value was significantly associated with the primary outcome (HR 1.09; P=.003).
- Definite CS patients had a greater need for heart transplantation or left ventricular assist device implantation, but no significant difference in all-cause mortality was observed compared to probable CS patients.
Conclusions:
- Both definite and probable cardiac sarcoidosis patients exhibit a high risk for sustained ventricular arrhythmias/sudden cardiac death and all-cause mortality.
- Despite similar risks for initial life-threatening events, definite CS is associated with a greater overall arrhythmia burden.
- The findings underscore the need for proactive management strategies for both diagnostic categories of CS to mitigate arrhythmic complications.
Background:
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.
Objective:
The purpose of this study was to evaluate the electrophysiological/arrhythmic phenotype and outcomes of patients with definite and probable CS.
Methods:
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.
Results:
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.
Conclusion:
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.
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