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Pathophysiology and clinical management of cardiac sarcoidosis

Nature Reviews Cardiology volume 12, pages 278–288 (2015)Cite this article

Subjects

Key Points

  • Cardiac sarcoidosis is characterized by formation of granulomas in the heart, and presentation can range from asymptomatic with an abnormal imaging scan to sudden cardiac death

  • Atrial arrhythmias are increasingly recognized as a clinical manifestation of cardiac sarcoidosis

  • Screening for cardiac sarcoidosis has not been standardized, but usually involves medical history and physical examination, electrocardiogram, and Holter monitoring or echocardiogram

  • Management of cardiac sarcoidosis involves both immunosuppressive therapy for the treatment of sarcoidosis and cardiac-specific therapy to treat ventricular dysfunction and heart rhythm disturbances

Abstract

Cardiac sarcoidosis is a potentially life-threatening condition characterized by formation of granulomas in the heart, resulting in conduction disturbances, atrial and ventricular arrhythmias, and ventricular dysfunction. The presentation of cardiac sarcoidosis ranges from asymptomatic with an abnormal imaging scan, to palpitations, syncope, symptoms of congestive heart failure, and sudden cardiac death. Screening for cardiac sarcoidosis has not been standardized, but the presence of cardiac symptoms on medical history and physical examination, and an abnormal electrocardiogram (ECG), Holter monitoring, or echocardiogram has been shown to be highly sensitive for detecting cardiac sarcoidosis. A signal-averaged ECG might also have a role in screening for cardiac sarcoidosis in asymptomatic patients. Although endomyocardial biopsies are highly specific for the diagnosis of cardiac sarcoidosis, procedural yield is very low and appropriate findings on cardiac MRI or PET are, therefore, often used as diagnostic surrogates. Treatment for cardiac sarcoidosis usually involves immunosuppressive therapy, particularly corticosteroids. Additional therapy might be required, depending on the clinical presentation, including implantation of an internal defibrillator, antiarrhythmic agents, and catheter ablation.

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Figure 1
Figure 2: An algorithm used to screen patients with sarcoidosis for cardiac involvement.
Figure 3: Cardiac MRI with evidence of delayed enhancement.
Figure 4: Pattern of uptake on an 18F-fluorodeoxyglucose PET scan.
Figure 5: Electroanatomical mapping of the right ventricle.

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Authors and Affiliations

  1. Division of Environmental and Occupational Health Science, National Jewish Health, Denver, 80206, CO, USA

    Nabeel Hamzeh

  2. Division of Cardiology, Albany Medical College, New York, 12208, Albany, NY, USA

    David A. Steckman

  3. Division of Pulmonary and Critical Care Medicine, Albany Medical College, New York, 12208, Albany, NY, USA

    Marc A. Judson

  4. Cardiac Electrophysiology & Arrhythmia Clinic, University of Colorado, Aurora, 80045, CO, USA

    William H. Sauer

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Hamzeh, N., Steckman, D., Sauer, W. et al. Pathophysiology and clinical management of cardiac sarcoidosis. Nat Rev Cardiol 12, 278–288 (2015). https://doi.org/10.1038/nrcardio.2015.22

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