The Heart Rhythm Society (HRS) released the Expert Consensus Statement on the Diagnosis and Management of Arrhythmias Associated with Cardiac Sarcoidosis. The expert consensus statement presented today at Heart Rhythm 2014, the Heart Rhythm Society’s 35th Annual Scientific Sessions, provides the first internationally accepted recommendations on the proper diagnosis and management of arrhythmias associated with cardiac sarcoidosis (CS). The document is intended to address the challenges faced by clinicians caring for patients with CS, a very rare and complex disease.
CS is an inherited immune system disorder of unknown etiology. Noncaseating granulomas, an inflammation that typically occurs when the immune system attempts to wall off unknown substances, are the pathological hallmark and are most often associated with pulmonary involvement but may also involve the heart, liver, peripheral lymph node, spleen, skin, eyes, phalangeal bones, parotid gland, or other organs and tissues. The annual incidence of sarcoidosis in the United States has been estimated at 10.9 per 100,000 in whites and 35.5 per 100,000 in African Americans . It is most common in patients that are 25-45 years old, and more common in women than men. Currently, the disease is challenging to diagnose and there is no easy way to assess disease activity or severity .
The consensus reports the guidance and recommendations of a writing group comprised of international experts. As the consensus recommendations are addressing a rare disease, there are no randomized or blinded studies. Therefore, the recommendations summarize the opinions of the expert writing group based on data derived from case series and registries that have followed patients and recorded outcome information.
The statement includes the following guidance and recommendations for cardiologists and cardiac electrophysiologists (EPs):
- Criteria for the diagnosis of CS through two primary pathways
- Screening for cardiac involvement in patients with biopsy-proven extracardiac sarcoidosis
- Screening for CS on patients with specific cardiac presentations
- Management of conduction abnormalities
- Management of atrial and ventricular arrhythmias associated with CS
- Risk stratification for sudden cardiac death in CS
- Appropriate indications for implantable cardioverter-defibrillator (ICD) implantation, including timing, specific complications and programming
The document establishes working criteria for the diagnosis of CS on the basis of expert opinion and limited, available data. The authors identified key areas in which data are lacking to help guide future collaborative research efforts. Specifically, the authors of the consensus statement strongly encourage a multicenter collaborative approach to study CS to further inform the proper management of patients with this complex disease.
“The complexity of cardiac sarcoidosis makes it challenging for clinicians to diagnose and manage treatment. This document provides much needed guidance for both primary care physicians and cardiologists who are looking for best practices and clinical consensus when treating this rare disease,” said one of the lead authors David H. Birnie, MD, University of Ottawa Heart Institute. “Clinicians from around the world have come together to further advance our knowledge of this disease and it is important that we continue our research to ensure the best quality care is delivered.”
The expert panel was comprised of experts from organizations across the globe, including representatives from the American College of Cardiology (ACC), American College of Chest Physicians, American Heart Association (AHA), Asia Pacific Heart Rhythm Society (APHRS), European Heart Rhythm Association (EHRA), and World of Association for Sarcoidosis and Other Granulomatous Disorder (WASOG). The document will be published in the July edition of HeartRhythm.
“HRS Management of Arrhythmias Associated with Cardiac Sarcoidosis” [Friday, May 9, 2014, 8:00 a.m. – 9:30 a.m. PDT, Room 108]
Heart Rhythm 2014 is the most comprehensive educational program for heart rhythm professionals, featuring more than 8,000 attendees, 250 educational sections and more than 130 exhibitors showcasing innovative products and services. The Heart Rhythm Society’s Annual
Scientific Sessions have become the must-attend event of the year, allowing the exchange of new vital ideas and information among colleagues from every corner of the globe.
 Statement on sarcoidosis. Joint Statement of ATS, ERS and WASOG adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999;160:736-755.
 Newman LS, Rose CS, Maier LA. Sarcoidosis N Engl J Med 1997;336:1224-1234.