Outcomes of patients with definite and suspected isolated cardiac sarcoidosis treated with an implantable cardiac defibrillator.

Purpose: Cardiac sarcoidosis (CS) patients are at increased risk for sudden death. Isolated CS is rare and can be difficult to diagnose.
Methods: In this multicenter retrospective review, patients with CS and an implantable cardiac defibrillator (ICD) were identified.
Results: Of 235 patients with CS and ICD, 13 (5.5 %) had isolated CS, including 7 (3.0 %) with definite isolated CS (biopsy or necropsy-proven) and 6 (2.6 %) with suspected isolated CS based on a constellation of clinical, ECG, and imaging findings. Among 13 patients 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 cardiac magnetic resonance (CMR) (n = 2), biopsy (n = 3), CMR and biopsy (n = 2), CMR and positron emission tomography (PET) (n = 2), PET (n = 1), late enhanced cardiac CT (n = 1), pathology at heart transplant (n = 1), and autopsy (n = 1). 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 %) with sarcoidosis in other organs (p = 0.04). Over a mean of 4.2 years, 9 of 13 (69.2 %) patients with isolated CS received appropriate ICD therapy, including anti-tachycardia pacing (ATP) and/or shock, compared with 75 of 222 (33.8 %) patients with cardiac and extracardiac sarcoidosis (p = 0.0150). Six of 7 (85.7 %) patients with definite isolated CS received appropriate ICD intervention, compared with 78 of 228 patients (34.2 %) without definite isolated CS (p = 0.0192.)
Conclusions: In this retrospective study, patients with isolated CS had very high rates of appropriate ICD therapy. Prospective, long-term follow-up of consecutive patients with isolated CS is needed to determine the true natural history and rates of ventricular arrhythmias in this rare and difficult-to-diagnose disease.