title: Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy.
abstract: OBJECTIVE
  The aim of this study was to evaluate the survival of patients with hypertrophic cardiomyopathy (HCM) after resuscitated ventricular fibrillation or syncopal sustained ventricular tachycardia (VT/VF) when treated with low dose amiodarone or implantable cardioverter defibrillators (ICDs).
  
  
  BACKGROUND
  Prospective data on clinical outcome in patients with HCM who survive a cardiac arrest are limited, but studies conducted before the widespread use of amiodarone and/or ICD therapy suggest that over a third die within seven years from sudden cardiac death or progressive heart failure.
  
  
  METHODS
  Sixteen HCM patients with a history of VT/VF (nine male, age at VT/VF 19 +/- 8 years [range 10 to 36]) were studied. Syncopal sustained ventricular tachycardia/ventricular fibrillation occurred during or immediately after exertion in eight patients and was the initial presentation in eight. One patient had disabling neurologic deficit after VT/VF. Before VT/VF, two patients had angina, four had syncope and six had a family history of premature sudden cardiac death. After VT/VF all patients were in New York Heart Association class I or II, three had nonsustained VT during ambulatory electrocardiography and 11 had an abnormal exercise blood pressure response. After VT/VF eight patients were treated with low dose amiodarone and six received an ICD. Prophylactic therapy was declined by two patients.
  
  
  RESULTS
  Mean follow-up was 6.1 +/- 4.0 years (range 0.5 to 14.5). Cumulative survival (death or ICD discharge) for the entire cohort was 59% at five years (95% confidence interval: 33% to 84%). Thirteen (81%) patients were alive at last follow-up. Two patients died suddenly while taking low dose amiodarone, and one died due to neurologic complications of his initial cardiac arrest. Three patients had one or more appropriate ICD discharges during follow-up; the times to first shock after ICD implantation were 23, 197 and 1,124 days.
  
  
  CONCLUSIONS
  This study shows that patients with HCM who survive an episode of VT/VF remain at risk for a recurrent event. Implantable cardioverter defibrillator therapy appears to offer the best potential benefit regarding outcome.
authors.affiliation: Department of Cardiological Sciences, St. George's Hospital Medical School, London, United Kingdom.
authors.forename: P M
authors.initials: PM
authors.lastname: Elliott
authors.forename: S
authors.initials: S
authors.lastname: Sharma
authors.forename: A
authors.initials: A
authors.lastname: Varnava
authors.forename: J
authors.initials: J
authors.lastname: Poloniecki
authors.forename: E
authors.initials: E
authors.lastname: Rowland
authors.forename: W J
authors.initials: WJ
authors.lastname: McKenna
mesh_terms: D000293:Adolescent; D000328:Adult; D000638:Amiodarone / Q000008:administration & dosage; D000889:Anti-Arrhythmia Agents / Q000008:administration & dosage; D002312:Cardiomyopathy, Hypertrophic / Q000235:genetics / Q000401:mortality* / Q000628:therapy; D002648:Child; D004252:DNA Mutational Analysis; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000517:prevention & control; D017147:Defibrillators, Implantable; D004305:Dose-Response Relationship, Drug; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005820:Genetic Testing; D006323:Heart Arrest / Q000235:genetics / Q000401:mortality* / Q000517:prevention & control; D006801:Humans; D008297:Male; D011446:Prospective Studies; D012151:Resuscitation; D012518:Sarcomeres / Q000235:genetics; D015996:Survival Rate; D017180:Tachycardia, Ventricular / Q000235:genetics / Q000401:mortality* / Q000628:therapy; D014693:Ventricular Fibrillation / Q000235:genetics / Q000401:mortality / Q000628:therapy
chemical_list: D000889:Anti-Arrhythmia Agents; D000638:Amiodarone