Strategies for Managing Arrhythmias in Patients with Cardiac Sarcoidosis

Abstract

Sarcoidosis is a systemic granulomatous disease that frequently involves the myocardium. Unfortunately, the sentinel manifestations of cardiac sarcoidosis are often potentially fatal bradyarrhythmia and tachyarrhythmia. Advanced imaging modalities such as cardiac magnetic resonance have allowed for increased diagnosis of cardiac involvement. The current review article explores diagnosis and treatment strategies for arrhythmias in patients with cardiac sarcoidosis.

Keywords: Arrhythmia; heart block; sarcoidosis; sudden death.

Figure 1:

Noncaseating granuloma obtained from an endomyocardial biopsy of a patient with sarcoidosis, thereby confirming cardiac involvement.

Figure 2:

CMR scans of four patients with sarcoidosis and evidence of cardiac involvement. Both noncontrast (top row) and LGE (bottom row) images are shown. A: Patient 1 demonstrates evidence of myocardial fibrosis with delayed enhancement of the interventricular septum and inferior and lateral walls (arrows). B: Patient 2 demonstrates a noncoronary-artery-disease pattern of delayed hyperenhancement with involvement of the right ventricular aneurysm, basal inferior septum, and lateral wall of the left ventricle. C: Patient 3 demonstrates evidence of focal midmyocardial scar in the region of the His-Purkinje system and the basal inferior septum. D: Finally, patient 4 has evidence of diffuse involvement.

Figure 3:

A: Delayed hyperenhancement of a patient with cardiac sarcoidosis demonstrates diffuse, patchy involvement in areas throughout the right and left ventricles (arrows). B: FDG-PET demonstrates active inflammation in areas that correspond to areas of LGE. Courtesy of Steven R. Sigman, MD.

Figure 4:

2017 AHA/ACC/HRS recommendations for the prevention of sudden cardiac death in patients with cardiac sarcoidosis. *ICD candidacy as determined by functional status, life expectancy, or patient preference. †For recurrent sustained monomorphic VT, refer to Figure 2. Reproduced with permission from: Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2017 Oct 25. pii: S0735-1097(17)41306-4. [Epub ahead of print]. EP: electrophysiology; GDMT: guidelines-directed management and therapy; ICD: implantable cardioverter-defibrillator; LVEF: left ventricular ejection fraction; MRI: magnetic resonance imaging; PET: positron-emission tomography; SCA: sudden cardiac arrest; SCD: sudden cardiac death; VA: ventricular arrhythmia; VT: ventricular tachycardia.