William M. Miles, MD, FHRS University of Florida College of Medicine Gainesville, FL ICD Implantation: Genetic Arrhythmia Syndromes

Genetic Arrhythmia Syndromes  Long- and short-QT syndromes  Brugada syndrome  Idiopathic VF  Catecholaminergic polymorphic VT Most of these patients have no evidence of structural heart disease or LV dysfunction, and long-term prognosis is excellent if the arrhythmia is controlled.

Indications for ICD Implantation: Genetic Arrhythmia Syndromes ICD may be considered (Class IIb)  Primary prevention Patients with long QT syndrome and risk factors for SCD  e.g. markedly prolonged QTc or T wave alternans, “malignant” mutation

Indications for ICD Implantation: Genetic Arrhythmia Syndromes ICD implantation is indicated (Class I)  Secondary prevention Survivors of cardiac arrest after exclusion of completely reversible causes ICD implantation is reasonable (Class IIa)  Secondary prevention Patients with long-QT syndrome who are having syncope and/or VT while receiving beta blockers Patients with Brugada syndrome who have had Syncope Documented VT that has not resulted in cardiac arrest Patients with catecholaminergic polymorphic VT with syncope and/or sustained VT while receiving beta blockers