Abstract 12066: Patients With Isolated Cardiac Sarcoidosis and Implantable Cardiac Defibrillators Have High Rates of Ventricular Arrhythmias
Introduction: Cardiac sarcoidosis (CS) patients are at increased risk for sudden death from ventricular arrhythmias. Isolated CS is rare and can be difficult to diagnose.
Hypothesis: We tested the hypothesis that patients with isolated CS are at high risk for ventricular arrhythmias.
Methods: In this multicenter retrospective data review, electrophysiologists at academic medical centers identified patients with CS and an implantable cardiac defibrillator (ICD). Data were collected including extracardiac sarcoidosis organ involvement and ventricular arrhythmias on ICD interrogation.
Results: Of 235 patients with CS and an ICD, 13 (5.5%) had isolated CS. Of 13 with isolated CS, 10 (76.9%) were male, mean age was 53.8 ± 7.6 years, and mean left ventricular ejection fraction was 38.3 ± 16.5. Diagnosis was made by MRI (n=3), biopsy (n=3), MRI and biopsy (n=2), MRI and PET (n=1), PET (n=1), cardiac CT (n=1), pathology at time of heart transplant (n=1) and autopsy (n=1). Eleven (84.6%) had an abnormal ECG, including 1st degree AV block (n = 2), 3rd degree AV block (n=1), right bundle branch block with left anterior fascicular block (n=4), left bundle branch block (n=1), and premature ventricular contractions (n=2). Of 6 patients with isolated CS who had a cardiac MRI, 6 (100%) were abnormal, and 5 of 7 (71.4%) patients had an abnormal endomyocardial biopsy. Eight of 13 (61.5%) patients with isolated CS had a secondary prevention indication (VT in 6 and VF in 2) vs. 80 of 222 (36.0%) patients with sarcoid in other organs (p = 0.04). Patients with isolated CS were more likely to be on an antiarrhythmic medication (84.6%) vs. those with extracardiac disease (41.6%) (p = 0.0030). Over a mean of 4.2 years, 9 of 13 (69.2%) of patients with isolated CS received appropriate ICD therapy, including ATP and/or shock, compared with 75 of 222 (33.8%) patients with extracardiac sarcoidosis (P = 0.0064). Eight of 13 (61.5%) patients with isolated CS received an appropriate ICD shock vs. 59 of 222 (26.6%) patients with extracardiac sarcoidosis (p = 0.0035).
Conclusions: Patients with isolated CS and an ICD have very high rates of ventricular arrhythmias, with 69% receiving an appropriate shock over 4 years. To our knowledge, this is the first evaluation of ICD efficacy in patients with isolated CS.
- © 2013 by American Heart Association, Inc.