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Whilst the decision regarding defibrillator implantation in a patient with a familial sudden cardiac death syndrome is likely to be most significant for any particular individual, the clinical decision-making process itself is complex and requires interpretation and extrapolation of information from a number of different sources. This document provides recommendations for adult patients with the congenital Long QT syndromes, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy. Although these specific conditions differ in terms of clinical features and prognosis, it is possible and logical to take an approach to determining a threshold for implantable cardioveter-defibrillator implantation that is common to all of the familial sudden cardiac death syndromes based on estimates of absolute risk of sudden death.

Original publication

DOI

10.1093/europace/euq261

Type

Journal article

Journal

Europace

Publication Date

08/2010

Volume

12

Pages

1156 - 1175

Keywords

Adult, Arrhythmias, Cardiac, Cardiomyopathies, Death, Sudden, Cardiac, Defibrillators, Implantable, Humans, United Kingdom