Screening for, and management of, possible arrhythmogenic syndromes (channelopathies/ion channel diseases).
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.
Svendsen
Jesper Hastrup
JH
Department of Cardiology, The Heart Centre, Rigshospitalet, University of Copenhagen and The Danish National Research Foundation Centre for Cardiac Arrhythmia, Denmark. hastrup@rh.dk
Geelen
Peter
P
D001145:Arrhythmias, Cardiac / Q000175:diagnosis* / Q000503:physiopathology*; D053840:Brugada Syndrome / Q000175:diagnosis / Q000503:physiopathology; D053447:Channelopathies / Q000175:diagnosis* / Q000503:physiopathology*; D003625:Data Collection; D022062:Electrophysiologic Techniques, Cardiac; D005060:Europe; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000503:physiopathology; D008403:Mass Screening / Q000639:trends*; D008991:Monitoring, Physiologic; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000503:physiopathology