Screening for, and management of, possible arrhythmogenic syndromes (channelopathies/ion channel diseases)

Abstract

This survey assesses the current management strategies for individuals with electrocardiographic features, suggesting an arrhythmogenic syndrome [including long QT syndrome (LQTS), Brugada syndrome (BS), catecholaminergic polymorphic ventricular tachycardia (CPVT) or short QT syndrome] or family members of patients with a known arrhythmogenic syndrome, in 44 large European centres. The principal findings of this survey were: (i) The number of new patients with arrhythmogenic syndromes (symptomatic and asymptomatic) is relatively small; (ii) The clinical work-up of these patients consists mainly of non-invasive tests; (iii) A relatively high use of genetic testing is noted, especially in LQTS and CPVT; (iv) EP testing is commonly performed in asymptomatic BS patients and in family members of symptomatic BS patients; and (v) The majority of European electrophysiologists focus on first-degree relatives when dealing with family members of an index patient. © The Author 2010.