Atrial Arrhythmias in Clinically Manifest Cardiac Sarcoidosis: Incidence, Burden, Predictors, and Outcomes

Abstract

Background Recent data have suggested a substantial incidence of atrial arrhythmias (AAs) in cardiac sarcoidosis (CS). Our study aims were to first assess how often AAs are the presenting feature of previously undiagnosed CS. Second, we used prospective follow-up data from implanted devices to investigate AA incidence, burden, predictors, and response to immunosuppression. Methods and Results This project is a substudy of the CHASM-CS (Cardiac Sarcoidosis Multicenter Prospective Cohort Study; NCT01477359). Inclusion criteria were presentation with clinically manifest cardiac sarcoidosis, treatment-naive status, and implanted with a device that reported accurate AA burden. Data were collected at each device interrogation visit for all patients and all potential episodes of AA were adjudicated. For each intervisit period, the total AA burden was obtained. A total of 33 patients met the inclusion criteria (aged 56.1±7.7 years, 45.5% women). Only 1 patient had important AAs as a part of the initial CS presentation. During a median follow-up of 49.1 months, 11 of 33 patients (33.3%) had device-detected AAs, and only 2 (6.1%) had a clinically significant AA burden. Both patients had reduced burden after CS was successfully treated and there was no residual fluorodeoxyglucose uptake on positron emission tomography scan. Conclusions First, we found that AAs are a rare presenting feature of clinically manifest cardiac sarcoidosis. Second, AAs occurred in a minority of patients at follow-up; the burden was very low in most patients. Only 2 patients had clinically significant AA burden, and both had a reduction after CS was treated. Registration URL: https://www.clini​caltr​ials.gov; unique identifier NCT01477359.

Keywords: atrial arrhythmia; atrial fibrillation; cardiac sarcoidosis.

Conflict of interest statement

D.J. has received consultant fees from AbbVie and Advanced Accelerator Applications. R.S.B. is or has been a consultant for and receives grant funding from GE Healthcare, Lantheus Medical Imaging, and Jubilant DraxImage. The remaining authors have no disclosures to report.

Figures

Figure 1. Clinical course of the 2 patients with more than low burden atrial arrhythmia (AA) at follow‐up after initial diagnosis of cardiac sarcoidosis (CS).

Device‐detected AA burden is shown on top, along with a summary of CS treatment and fluorodeoxyglucose (FDG)‐positron emission tomography (PET) activity below. A, A 56‐year‐old woman (patient 4) with a diagnosis of CS after presenting with complete heart block. There was no history of AA before her diagnosis and no atrial FDG uptake on her pretreatment PET scan. B, A 56‐year‐old woman (patient 5) with a diagnosis of CS after presenting with monomorphic ventricular tachycardia (VT). There was no history of arrhythmia before her diagnosis and there was biatrial FDG uptake on her pretreatment PET scan. AF indicates atrial fibrillation; MMF, mycophenolate mofetil; and MTX, methotrexate.

Figure 2. Fusion (left) and whole‐body fluorodeoxyglucose‐positron emission tomography (right) images of a 59‐year‐old patient who presented with complete heart block.

Note: focal uptake in the left atrium (maximum standardized uptake value, 4.43) and right atrium (maximum standardized uptake value, 5.54), shown by the green arrows. This patient has had no atrial arrhythmias in the 9 years since diagnosis.