title: Acute and chronic management in patients with Brugada syndrome associated with electrical storm of ventricular fibrillation.
abstract: BACKGROUND
  Some patients with Brugada syndrome experience an electrical storm of ventricular fibrillation (VF).
  
  
  OBJECTIVE
  The purpose of this study was to investigate the clinical, laboratory, electrocardiographic, and electrophysiologic characteristics, acute and subsequent chronic treatment, and follow-up data of patients with Brugada syndrome associated with electrical storm of VF.
  
  
  METHODS
  Sixty-seven patients with Brugada syndrome (65 men and 2 women, age 46 +/- 14 years) were divided into three groups: 7 patients with a history of electrical storm of VF (group I), 39 symptomatic patients with documented VF and/or syncope (group II), and 21 asymptomatic patients (group III). Electrical storm was defined as three or more episodes of VF per day recorded by the memory of an implantable cardioverter-defibrillator.
  
  
  RESULTS
  No significant differences were observed among the three groups with regard to clinical (age at diagnosis, familial history of sudden cardiac death), laboratory (SCN5A mutation and serum potassium level), electrocardiographic and electrophysiologic characteristics, and follow-up duration after diagnosis. However, arrhythmic events during follow-up after diagnosis and number of arrhythmic events per patient were significantly higher in group I compared with groups II and III. Isoproterenol infusion (0.003 +/- 0.003 microg/kg/min for 24 +/- 13 days) completely suppressed electrical storm of VF in all five patients treated and was successfully replaced with oral medications, including denopamine, quinidine, isoproterenol, cilostazol, and bepridil alone or in combination.
  
  
  CONCLUSIONS
  No specifically clinical, laboratory, electrocardiographic, and electrophysiologic characteristics were recognized in patients with Brugada syndrome associated with electrical storm of VF. Isoproterenol infusion was effective as an acute treatment in suppressing electrical storm of VF and was successfully replaced with chronic oral medications.
authors.affiliation: Division of Cardiology, Department of Internal Medicine, National Cardiovascular Center, Osaka, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Ohgo
authors.forename: Hideo
authors.initials: H
authors.lastname: Okamura
authors.forename: Takashi
authors.initials: T
authors.lastname: Noda
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Satomi
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Suyama
authors.forename: Takashi
authors.initials: T
authors.lastname: Kurita
authors.forename: Naohiko
authors.initials: N
authors.lastname: Aihara
authors.forename: Shiro
authors.initials: S
authors.lastname: Kamakura
authors.forename: Tohru
authors.initials: T
authors.lastname: Ohe
authors.forename: Wataru
authors.initials: W
authors.lastname: Shimizu
mesh_terms: D000208:Acute Disease; D000328:Adult; D000368:Aged; D053840:Brugada Syndrome / Q000150:complications / Q000188:drug therapy*; D002908:Chronic Disease; D016757:Death, Sudden, Cardiac / Q000517:prevention & control*; D005260:Female; D006801:Humans; D007545:Isoproterenol / Q000627:therapeutic use; D007564:Japan; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011802:Quinidine / Q000627:therapeutic use; D014693:Ventricular Fibrillation / Q000188:drug therapy* / Q000209:etiology
chemical_list: D011802:Quinidine; D007545:Isoproterenol