William M. Miles, MD, FHRS

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

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Indications for ICD ImplantationGenetic
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

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Indications for ICD ImplantationGenetic
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