EUH Hunt Conference: What is the diagnostic workup of cardiac sarcoidosis?

The Bottom Line: Cardiac sarcoidosis has varied presentations depending on the extent and location of cardiac granulomas. Patients may have AV block, heart failure, or sudden cardiac death. The disease should be considered especially in young or middle-aged women who present with heart failure and/or AV block or ventricular arrhythmias.

Echocardiography may show septal thinning, wall motion abnormalities in a noncoronary distribution, or LV aneurysm. Diagnosis can be made by EMB; however, given the patchy distribution of disease within the myocardium, the sensitivity is only 20–26%. Positron emission tomography and magnetic resonance imaging are both useful modalities for identifying cardiac sarcoidosis. Diagnosis requires a combination of clinical/imaging evidence of cardiac involvement along with tissue diagnosis of extracardiac sarcoid.

References: DynaMed Plus [Internet]. Ipswich (MA): EBSCO Information Services. 1995 – . Record No. 306338, Cardiac sarcoidosis; [updated 2017 Mar 31, cited 2017 December 19]; [about 11 screens]. Emory login required.

Selby, Van N.. “Myocarditis, Toxic Cardiomyopathy, and Stress Cardiomyopathy.” CURRENT Diagnosis & Treatment: Cardiology, 5e   Ed. Michael H. Crawford. New York, NY: McGraw-Hill.

Summary: According to DynaMed Plus (2017), to make the diagnosis:

  • suspect cardiac sarcoidosis in patients with extracardiac sarcoidosis plus
    • cardiac symptoms, such as palpitations, presyncope or syncope, or symptoms of heart failure (such as dyspnea)
    • cardiac manifestations, such as atrioventricular (AV) block, ventricular tachycardia, atrial arrhythmia, or supraventricular tachycardia
  • isolated cardiac sarcoidosis may be suspected in patients without extracardiac sarcoidosis but with cardiac manifestations of either
    • unexplained high-degree (Mobitz type II or third-degree) AV block
    • symptomatic left atrial tachycardia without history of ablation or surgery for atrial fibrillation
  • no formal diagnostic criteria for cardiac sarcoidosis have been established but 3 guidelines have proposed criteria, which generally include
    • histological diagnosis if noncaseating granulomas composed of epithelioid cells in myocardial tissue on endomyocardial biopsy
    • clinical diagnosis if all of
      • extracardiac sarcoidosis confirmed on histology in another organ
      • presence of ≥ 1 criteria for cardiac sarcoidosis
      • other causes of cardiac manifestation have been reasonably excluded