Implantable cardioverter defibrillator therapy in patients with arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, or no structural heart disease

Am Heart J. 1994 Apr;127(4 Pt 2):1151-8. doi: 10.1016/0002-8703(94)90103-1.


Recent technical developments in implantable cardioverter defibrillator (ICD) systems and reduced operative mortality and morbidity rates associated with ICD implantation have expanded the indications for ICD treatment of ventricular tachyarrhythmias. This review summarizes data regarding ICD therapy in patients with arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, and idiopathic ventricular fibrillation and presents preliminary concepts for identification of patients who will benefit more from ICD therapy than from pharmacologic and other nonpharmacologic approaches. Recent studies suggest that ICD therapy may improve long-term prognosis by reliably terminating recurrences of life-threatening arrhythmias. Appropriate ICD therapies during mean follow-up periods of 12 to 36 months occurred in 30% of patients with idiopathic ventricular fibrillation to 50% of patients with arrhythmogenic right ventricular cardiomyopathy and long QT syndrome. At present no strict recommendations can be given for ICD implantation in these patients. However, at least in cardiac arrest survivors in whom the clinical arrhythmia is not reproducibly inducible during electrophysiologic study, ICD therapy appears to be superior to other treatment options with regard to long-term survival and thus should be considered as a first-line treatment. We are hopeful that continued study of long-term follow-up with and without ICD treatment and improved risk stratification will lead to better criteria for selection of treatment options.

Publication types

  • Review

MeSH terms

  • Adult
  • Child
  • Clinical Trials as Topic
  • Defibrillators, Implantable*
  • Female
  • Follow-Up Studies
  • Heart Arrest / therapy
  • Humans
  • Hypertrophy, Right Ventricular / complications
  • Hypertrophy, Right Ventricular / therapy*
  • Long QT Syndrome / therapy*
  • Male
  • Risk Factors
  • Tachycardia, Ventricular / therapy*
  • Ventricular Fibrillation / therapy*