Diagnosis and management of patients with inherited arrhythmia syndromes in Europe: results of the European Heart Rhythm Association Survey

Abstract

Inherited arrhythmia disorders associated with structurally normal heart (i.e. long and short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, early repolarization syndrome, idiopathic ventricular fibrillation) cause 10% of 1.1 million sudden deaths in Europe and the USA. The purpose of this European Heart Rhythm Association (EHRA) electrophysiology wire survey was to assess the European clinical practice adopted for the diagnosis and management of these disorders. The survey was based on an electronic questionnaire sent out to the EHRA Research Network centres. Responses were received from 50 centres in 23 countries. The results of the survey show that inherited arrhythmia syndromes have a relatively low burden and are diagnosed and managed in accordance with the current guidelines. However, more than 50% of centres do not participate in any existing registry underlining the need for establishing a pan-European registry of these disorders.

Keywords: EHRA survey; EP wire; Genetic testing; Inherited arrhythmia syndrome; Ion channelopathy; Sudden death; Ventricular arrhythmia.