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Three Scientific Societies Collaborate to Release Expert Consensus Statement on Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes

Worldwide panel of experts presents the first comprehensive recommendations on patients with inherited arrhythmias at Heart Rhythm 2013

The complexity and prevalence of inherited cardiovascular diseases is growing, thereby creating a greater demand for the proper diagnosis and management of patients with these conditions.

Arrhythmias and Device Therapy

DENVER, May 10, 2013 – The Heart Rhythm Society (HRS), the European Heart Rhythm Association (EHRA) and Asia Pacific Heart Rhythm Society (APHRS), release the HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes. The expert consensus statement presented today at Heart Rhythm 2013, the Heart Rhythm Society’s 34th Annual Scientific Sessions, provides the first comprehensive statement of recommendations on the proper diagnosis and management of patients with inherited primary arrhythmia syndromes.  
The document addresses inherited conditions that can increase the risk of sudden cardiac death (SCD), especially in young individuals, as SCD is one of the leading causes of non-traumatic mortality in young individuals within Western Countries.  Long QT Syndrome (LQTS), a common inherited arrhythmia, causes about 3,000 to 4,000 sudden deaths in children and young adults each year in the United States, according to the National Heart, Lung, and Blood Institute.

“The complexity and prevalence of inherited cardiovascular diseases is growing, thereby creating a greater demand for the proper diagnosis and management of patients with these conditions,” said Silvia G. Priori, MD, PhD, Fondazione Salvatore Maugeri, Department of Molecular Medicine University of Pavia, Pavia, Italy, HRS Chairperson. “Clinicians and other healthcare professionals from around the world have come together to address this concern which is top of mind for our field.”

The consensus statement provides a state of the art review of the field and reports the recommendations of a writing group comprised of international experts. The recommendations summarize the opinions of the expert writing group, based on an extensive literature review as well as their own clinical experience. The consensus statement is for all healthcare professionals who manage the patient care of individuals who have survived cardiac arrest at a young age, family members of individuals who have died suddenly at a young age with a negative autopsy, patients in whom the diagnosis of an inherited arrhythmia is clinically possible, or young patients with unexplained syncope.
The statement provides the diagnostic risk stratification and management of patients, among others, affected by LQTS and Brugada Syndrome (BrS). Documented for the first time, the statement also includes a consensus of recommendations on the diagnosis, risk stratification, and management of the following arrhythmic diseases and unexplained causes of sudden death/cardiac arrest:

  • Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)
  • Short QT Syndrome (SQTS)
  • Early Repolarization (ER)
  • Progressive Cardiac Conduction Disease (PCCD)
  • Unexplained cardiac arrest including Idiopathic Ventricular Fibrillation (VF), Sudden Unexplained Death Syndrome (SUDS), and Sudden Unexplained Death in Infancy (SUDI)

“This document is a great advancement for electrophysiology because it provides one, comprehensive and uniform recommendation on all rare arrhythmia syndromes,” said Arthur A. Wilde, MD, PhD, University of Amsterdam, Amsterdam, Netherlands, EHRA Chairperson. “As new data emerges and our understanding of the role of genetics improves, collaboration like this will help ensure the best quality of care is delivered.”
An additional primary recommendation from the expert consensus writing committee is that patients and first-degree relatives with a diagnosed or suspected inherited cardiovascular disease should be evaluated in a dedicated clinic with appropriately trained clinical staff and the resources to provide support to the patient and family members.
The consensus statement has representatives on the writing committee from the American College of Cardiology Foundation (ACCF), the Association for European Pediatric and Congenital Cardiology (AEPC), the American Heart Association (AHA), and the Pediatric and Congenital Electrophysiology Society (PACES).  At the time of the release of this document, endorsement by these organizations is pending. The document will be published simultaneously in HeartRhythm and in EP Europace in fall 2013.

Sessions details:
“HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Inherited Arrhythmias” [Friday, May 10, 2012, 4:30 p.m. – 6:00 p.m. MDT, Room 108]

Heart Rhythm 2013 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.


  1. Carturan E, Basso C, Thiene G. [Molecular investigation of sudden death]. Giornale italiano di cardiologia (2006) 2007;8:752-9

Full paper

Notes to editor

About the Heart Rhythm Society
The Heart Rhythm Society is the international leader in science, education and advocacy for cardiac arrhythmia professionals and patients, and the primary information resource on heart rhythm disorders. Its mission is to improve the care of patients by promoting research, education and optimal health care policies and standards. Incorporated in 1979 and based in Washington, DC, it has a membership of more than 5,800 heart rhythm professionals in more than 72 countries around the world. For more information, visit, voted 2012’s “Best in Class” website for a nonprofit by the Interactive Media Council.
About the European Society of Cardiology (ESC)
The European Society of Cardiology (ESC) represents 80,000 cardiology professionals across Europe and the Mediterranean. Its mission is to reduce the burden of cardiovascular disease in Europe.
About EHRA
The European Heart Rhythm Association (EHRA) is a registered branch of the European Society of Cardiology. Its aim is to improve the quality of life of the European population by reducing the impact of cardiac arrhythmias and reduce sudden cardiac death. For more information, see or visit our stand n° 855 at HRS.
EHRA EUROPACE is an established, international conference attracting key opinion leaders, well-recognised scientists, physicians, allied professionals and industry, who all enjoy scientific exchange of the highest level in an informal atmosphere. This year, EHRA EUROPACE will take place from 23 to 26 June in Athens.