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Consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis
  1. David H Birnie1,
  2. William H Sauer2,
  3. Marc A Judson3
  1. 1Division of Cardiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada
  2. 2Section of Cardiac Electrophysiology, Division of Cardiology, University of Colorado, Aurora, Colorado, USA
  3. 3Division of Pulmonary and Critical Care Medicine, Albany Medical College, Albany, New York, USA
  1. Correspondence to Dr David H Birnie, Division of Cardiology, University of Ottawa Heart Institute, 40 Ruskin Street, Ottawa, ON K1Y 4W7, Canada; dbirnie{at}ottawaheart.ca

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Introduction

Recently, a consensus document on the diagnosis and management of cardiac sarcoidosis (CS) was published.1 This consensus statement was written by experts representing the Heart Rhythm Society, European Heart Rhythm Association, World Association for Sarcoidosis and other Granulomatous Disorders (WASOG), American College of Chest Physicians, American Heart Association the American College of Cardiology and the Asia Pacific Heart Rhythm Society.

The statement was a worldwide effort to address the clinical questions faced by physicians looking after patients with CS in the absence of conclusive clinical data. Although we consider that the recommendations will help in the care of these patients, it is perhaps only a foundation point for comprehending this poorly understood disease. Developing guidelines for uncommon conditions requires adapting the usual methodology for preparing these documents. Most obviously, there are limited randomised and/or blinded studies in the field. Therefore, all recommendations are level of evidence C (ie, expert opinions). The key recommendations are reviewed below.

Background

Sarcoidosis is a disease of unknown aetiology with non-caseating granulomas being the key pathological finding. The lungs are the most commonly affected organ but the disease may also variably occur in the heart, skin, liver, spleen, eye, peripheral lymph nodes and other organs. Recent data suggests that the disease is due to an unidentified antigenic trigger provoking an abnormal immunological response in genetically susceptible individuals. Sarcoidosis occurs worldwide, with the highest rates reported in African–American and Northern Europeans, particularly in women. The overall disease prevalence varies between about 4.7 and 64 in 100 000.

Clinically manifest cardiac involvement is reported in approximately 5% of patients with sarcoidosis. Features of CS are dependent on the activity, extent and location of the disease. The three main manifestations are (1) conduction abnormalities, (2) ventricular arrhythmias including unheralded sudden cardiac death and (3) heart failure. Also, it is …

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