OBJECTIVEClinical observation of electrophysiological study and implantable cardioverter defibrillator (ICD) therapy in patients with Brugada syndrome.

METHODSTen patients (all male) with Brugada wave (spontaneous or propafenone test positive in ECG) underwent electrophysiological study (EPS). The mean age was (41 +/- 10) years. They had no structural heart disease with echocardiogram and the angiogram work-up. The ICD implanted in the patients with EPS-induced ventricular fibrillation in those who were available.

RESULTSThree patients had the history of familial sudden cardiac death (SCD). Four patients had repeated syncope episodes, two of them had documented ventricular fibrillation during syncope episodes. The AH and HV intervals were 50 - 124 (86 +/- 21) ms and 41 - 84 (58 +/- 15) ms. The ventricular fibrillation was induced in four patients with syncope and atrioventricular reentry tachycardia in one patient with palpitation. Three patients had spontaneous or inducible atrial fibrillation. The ICD implanted in three patients with inducible ventricular fibrillation. Due to economic issue, one patient without ICD implantation had got SCD during follow-up. The patient with atrioventricular reentry tachycardia underwent a successful left atrioventricular accessory pathway ablation.

CONCLUSIONThe Brugada patients with syncope and high rate of inducible ventricular fibrillation in EPS are the high risk population for SCD, in whom ICD should implant promptly to prevent SCD.

Adult ; Brugada Syndrome ; physiopathology ; therapy ; Death, Sudden, Cardiac ; prevention & control ; Defibrillators, Implantable ; Electrophysiology ; Humans ; Male ; Middle Aged ; Ventricular Fibrillation ; therapy

Acute Coronary Syndrome ; therapy ; Angina, Unstable ; Arrhythmias, Cardiac ; Brugada Syndrome ; Cardiac Conduction System Disease ; Heart Conduction System ; abnormalities ; Humans ; Myocardial Infarction

The occurrence of ventricular fibrillation in the absence of any structural heart disease is classified as "primary electrical disorder". The paradigm of primary electrical disease is the long-QT syndrome. In 1992, Brugada and Brugada first reported a unique electrocardiographic syndrome in which ventricular fibrillation could occur without obvious structural heart disease. Their report drew attention to this condition as another form of primary electrical disorder and Brugada syndrome has subsequently been recognized in virtually all parts of the world. Brugada syndrome is electrocardiographically characterized by ST-segment elevation in the right precordial leads, either with or without right bundle branch block. Although its incidence and distribution have not been confirmed, it may be considerably more common in Southeast Asia. Sudden death is common, may be the first manifestation of disease during its clinical course, and is not effectively prevented by anti-arrhythmic drug therapy. This has led to the recommendation for the placement of an implantable cardioverter-defibrillator in symptomatic patients. The purpose of this paper is to describe the current understanding of Brugada syndrome.
Asia, Southeastern ; Brugada Syndrome* ; Bundle-Branch Block ; Death, Sudden ; Death, Sudden, Cardiac ; Defibrillators, Implantable ; Drug Therapy ; Electrocardiography ; Heart Diseases ; Humans ; Incidence ; Ventricular Fibrillation

Brugada syndrome is an arrhythmic syndrome characterized by right bundle branch block, ST segment elevation in the precordial lead (V1-V3), and sudden death caused by ventricular fibrillation, which is not effectively prevented by anti-arrhythmic drug therapy. We are reporting a 30-year-old male patient with Brugada syndrome who got an exploratory laparotomy and a tenorrhaphy due to stab wound which was managed with general anesthesia and brachial plexus block without any complications.
Adult ; Anesthesia, General ; Brachial Plexus Block* ; Brachial Plexus* ; Brugada Syndrome* ; Bundle-Branch Block ; Death, Sudden ; Drug Therapy ; Humans ; Laparotomy ; Male ; Ventricular Fibrillation ; Wounds, Stab

Brugada syndrome describes a subgroup of patients at risk of ventricular fibrillation, but with no evidence of an underlying structural heart disease associated with an ECG pattern of right bundle branch block and a ST segment elevation in the right precordial leads (V1 to V3). This syndrome is familial with an autosomal dominant mode of transmission, and may be considerably more common in Southeast Asia. It is associated with a significant risk of ventricular tachyarrhythmias and sudden death, which is not effectively prevented by anti-arrhythmic drug therapy. We experienced such a case under general anesthesia for a hemi-colectomy, in a 32-year-old male patient diagnosed as having Brugada syndrome by characteristic ECG findings without untoward cardiovascular events.
Adult ; Anesthesia, General ; Asia, Southeastern ; Brugada Syndrome* ; Bundle-Branch Block ; Death, Sudden ; Drug Therapy ; Electrocardiography ; Heart Diseases ; Humans ; Male ; Tachycardia ; Ventricular Fibrillation

Brugada syndrome describes a subgroup of patients at risk of ventricular fibrillation, but with no evidence of an underlying structural heart disease associated with an ECG pattern of right bundle branch block and a ST segment elevation in the right precordial leads (V1 to V3). This syndrome is familial with an autosomal dominant mode of transmission, and may be considerably more common in Southeast Asia. It is associated with a significant risk of ventricular tachyarrhythmias and sudden death, which is not effectively prevented by anti-arrhythmic drug therapy. We experienced such a case under general anesthesia for a hemi-colectomy, in a 32-year-old male patient diagnosed as having Brugada syndrome by characteristic ECG findings without untoward cardiovascular events.
Adult ; Anesthesia, General ; Asia, Southeastern ; Brugada Syndrome* ; Bundle-Branch Block ; Death, Sudden ; Drug Therapy ; Electrocardiography ; Heart Diseases ; Humans ; Male ; Tachycardia ; Ventricular Fibrillation

PURPOSE: Our study aims to analyze prognosis after implantable cardioverter-defibrillator (ICD) implantation in Korean patients with Brugada syndrome (BrS). MATERIALS AND METHODS: This was a retrospective study of BrS patients implanted with an ICD at one of four centers in Korea between January 1998 and April 2012. Sixty-nine patients (68 males, 1 female) were implanted with an ICD based on aborted cardiac arrest (n=38, 55%), history of syncope (n=17, 25%), or induced ven tricular tachyarrhythmia on electrophysiologic study in asymptomatic patients (n=14, 20%). A family history of sudden cardiac death and a spontaneous type 1 electrocardiography (ECG) were noted in 13 patients (19%) and 44 patients (64%), respectively. RESULTS: During a mean follow-up of 59+/-46 months, 4.6+/-5.5 appropri ate shocks were delivered in 19 patients (28%). Fourteen patients (20%) experienced 5.2+/-8.0 inappropriate shocks caused by supraventricular arrhythmia, lead failure, or abnormal sensing. Six patients were admitted for cardiac causes during follow-up, but no cardiac deaths occurred. An episode of aborted cardiac arrest was a significant predictor of appropriate shock, and the composite of cardiac events in the Cox pro portional hazard model [hazard ratio (95% confidence interval) was 11.34 (1.31-97.94) and 4.78 (1.41-16.22), respectively]. However, a spontaneous type 1 ECG was not a predictor of cardiac events. CONCLUSION: Appropriate shock (28%) and inappropriate shock (20%) were noted during a mean follow-up of 59+/-46 months in Korean BrS patients implanted with an ICD. An episode of aborted cardiac ar rest was the most powerful predictor of cardiac events.
Adult ; Brugada Syndrome/*therapy ; Death, Sudden, Cardiac ; *Defibrillators, Implantable ; Electrocardiography ; Female ; Humans ; Male ; Middle Aged ; Prognosis ; Republic of Korea ; Retrospective Studies ; Treatment Outcome

Brugada syndrome can be unmasked by several conditions including a febrile state, marked leukocytosis, and electrolyte disturbances. Herein, we describe a 62-year-old man with cholangiocarcinoma in the first reported case of Brugada syndrome onset following photodynamic therapy.
Bile Duct Neoplasms/*drug therapy ; *Bile Ducts, Intrahepatic ; Brugada Syndrome/diagnosis/*etiology/therapy ; Cardiopulmonary Resuscitation ; Cholangiocarcinoma/*drug therapy ; Electrocardiography ; Fatal Outcome ; Fever/etiology ; Humans ; Klatskin's Tumor/*drug therapy ; Male ; Middle Aged ; Photochemotherapy/*adverse effects ; Predictive Value of Tests ; Treatment Outcome

INTRODUCTIONBrugada syndrome (BrS) is a common genetic cause of sudden cardiac arrest (SCA) due to polymorphic ventricular tachycardia and ventricular fibrillation. The current recommended therapy for high-risk BrS patients is the use of an implantable cardioverter defibrillator (ICD). The present study aimed to report the clinical characteristics and treatment outcomes of BrS patients in northeastern Thailand.

METHODSPatients who were diagnosed with BrS or had a Brugada electrocardiogram (ECG) between 2005 and 2012 at Khon Kaen University's hospitals were enrolled in the present study. Patients' clinical characteristics, ECG type, laboratory results and treatment were reviewed.

RESULTSA total of 90 eligible patients were enrolled. Of these, 79 (87.8%) patients were symptomatic--65 (82.3%) had documented SCA and 14 (17.7%) had unexplained syncope. The remaining 11 (12.2%) patients were asymptomatic with Brugada ECG. A majority of the patients enrolled were born in northeastern Thailand. The mean age of the symptomatic patients was 44.49 ± 8.55 years. Among the symptomatic patients, a majority were male (n = 77, 97.5%) and 23 (29.1%) patients had a family history of SCA. Almost all BrS patients who were symptomatic (96.2%) received ICD treatment for secondary prevention. The number of patients who received appropriate ICD therapy was 4.2 times of those who received inappropriate shocks. Only 3 (3.8%) symptomatic BrS patients refused ICD treatment.

CONCLUSIONClinical characteristics did not distinguish between symptomatic BrS patients and asymptomatic patients with Brugada ECGs. The clinical characteristics and treatment outcomes for the symptomatic BrS patients with SCA and unexplained syncope were similar. Among the BrS patients implanted with secondary prevention ICD in Northeastern Thailand, nearly one-third had received appropriate ICD therapy, far exceeding the incidence of device-related complications and inappropriate therapy.

Adult ; Brugada Syndrome ; diagnosis ; therapy ; Death, Sudden, Cardiac ; prevention & control ; Defibrillators, Implantable ; Electrocardiography ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Secondary Prevention ; Syncope ; therapy ; Tachycardia, Ventricular ; prevention & control ; Thailand ; Ventricular Fibrillation ; prevention & control

Brugada syndrome is a rare channelopathy associated with the SCN5A gene that causes fatal ventricular arrhythmias. This case of Brugada syndrome, in which ventricular tachycardia (VT) was provoked by high fever, is the first report in a Korean child. The boy had retinoblastoma of his left eye diagnosed at 16 months of age. After chemotherapy, he contracted a catheter-related infection with a high fever up to 41degrees C leading to monomorphic VT. This was characterized as having right bundle branch block morphology, superior axis deviation, and a heart rate of 212/min. Direct current cardioversion recovered the VT to sinus rhythm after a lack of response to amiodarone and lidocaine. A second attack of VT that was not controlled by cardioversion, however, responded to lidocaine. The baseline electrocardiogram showed a long PR interval and QRS duration, and the patient's grandfather had a history of Brugada syndrome. A mutation in SCN5A was identified in this patient, his father, and his grandfather. The patient was treated with quinidine and followed up for 1 year.
Amiodarone ; Arrhythmias, Cardiac ; Axis, Cervical Vertebra ; Brugada Syndrome* ; Bundle-Branch Block ; Catheter-Related Infections ; Channelopathies ; Child ; Drug Therapy ; Electric Countershock ; Electrocardiography ; Fathers ; Fever ; Heart Rate ; Humans ; Lidocaine ; Male ; Quinidine ; Retinoblastoma ; Tachycardia, Ventricular*