Semin Respir Crit Care Med 2017; 38(04): 477-498
DOI: 10.1055/s-0037-1602381
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Cardiac Involvement in Sarcoidosis: Evolving Concepts in Diagnosis and Treatment

David M. Sayah1, Jason S. Bradfield2, John M. Moriarty3, John A. Belperio1, Joseph P. Lynch III1
  • 1Division of Pulmonary and Critical Care Medicine, Clinical Immunology, and Allergy, David Geffen School of Medicine at UCLA, Los Angeles, California
  • 2Division of Cardiology, Department of Medicine, UCLA Cardiac Arrhythmia Center, David Geffen School of Medicine at UCLA, Los Angeles, California
  • 3Division of Interventional Radiology, Department of Radiology, David Geffen School of Medicine at UCLA, Los Angeles, California
Further Information

Publication History

Publication Date:
27 July 2017 (online)

Abstract

Clinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart, with protean manifestations. Prognosis of cardiac sarcoidosis is related to the extent and site(s) of involvement. Most deaths due to cardiac sarcoidosis are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may be lethal. The definitive diagnosis of isolated cardiac sarcoidosis is difficult. The yield of endomyocardial biopsies is low; treatment of cardiac sarcoidosis is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Currently, 18F-fluorodeoxyglucose–positron emission tomography/computed tomography and gadolinium-enhanced magnetic resonance imaging scans are the key imaging modalities to diagnose cardiac sarcoidosis. The prognosis of cardiac sarcoidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high. Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy.