Acute and chronic management in patients with Brugada syndrome associated with electrical storm of ventricular fibrillation.
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.
Ohgo
Takeshi
T
Division of Cardiology, Department of Internal Medicine, National Cardiovascular Center, Osaka, Japan.
Okamura
Hideo
H
Noda
Takashi
T
Satomi
Kazuhiro
K
Suyama
Kazuhiro
K
Kurita
Takashi
T
Aihara
Naohiko
N
Kamakura
Shiro
S
Ohe
Tohru
T
Shimizu
Wataru
W
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
D011802:Quinidine; D007545:Isoproterenol