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Heart Rhythm Society

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Inherited arrhythmia syndromes increasing, says report

A comprehensive statement of recommendations on the proper diagnosis and management of patients with inherited primary arrhythmia syndromes was presented recently at the Heart Rhythm Society’s 34th Annual Scientific Sessions, held in Denver, Colorado (USA).

The HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes was released by the Heart Rhythm Society (HRS), the European Heart Rhythm Association (EHRA) and Asia Pacific Heart Rhythm Society (APHRS).

“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 HRS Chairperson Dr Silvia G Priori (Fondazione Salvatore Maugeri, Department of Molecular Medicine, University of Pavia, Pavia, Italy). “Clinicians and other healthcare professionals from around the world have come together to address this concern which is top of mind for our field,” she added.

Expert Consensus 2

Dr Silvia G Priori (University of Pavia, Italy)

The consensus statement provides recommendations from an international writing group, based on literature review and their own clinical experience. The consensus statement is aimed at all healthcare professionals who manage the 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 long QT Syndrome (LQTS) and Brugada Syndrome (BrS). 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 repolarisation (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 EHRA Chairperson Dr Arthur Wilde (University of Amsterdam, Amsterdam, Netherlands). “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,” he added.

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 document will be published simultaneously in HeartRhythm and in EP Europace in autumn 2013.

Published on: May 22, 2013

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