Age of First Arrhythmic Event in Brugada Syndrome: Data From the SABRUS (Survey on Arrhythmic Events in Brugada Syndrome) in 678 Patients (original) (raw)
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Heart rhythm, 2018
Detailed information on the profile of patients with Brugada syndrome (BrS) presenting their first arrhythmic event (AE) after prophylactic implantation of an implantable cardioverter-defibrillator (ICD) is limited. The objectives of this study were (1) to compare clinical, electrocardiographic, electrophysiologic, and genetic profiles of patients who exhibited their first documented AE as aborted cardiac arrest (group A) with profiles of those in whom the AE was documented after prophylactic ICD implantation (group B) and (2) to characterize group B patients' profile using the class II indications for ICD implantation established by HRS/EHRA/APHRS expert consensus statement in 2013. A survey of 23 centers from 10 Western and 4 Asian countries enabled data collection of 678 patients with BrS who exhibited their AE (group A, n = 426; group B, n = 252). The first AE occurred in group B patients 6.7 years later than in group A (mean age 46.1 ± 13.3 years vs 39.4 ± 15.1 years; P <...
Risk Stratification in Patients With Brugada Syndrome Without Previous Cardiac Arrest
Circulation Journal, 2015
recurrence rate of VF in patients with BrS who had previously experienced cardiac arrest. 3-7 Further discussion regarding the indications for implantable cardioverter defibrillator (ICD) in these patients (secondary prevention) is unnecessary. The indications for ICD for primary prevention of SCD in patients with BrS without a history of VF or cardiac arrest, however, have not been well evaluated. A recent consensus statement suggested the indication of ICD for these patients based on the following risk factors: spontaneous diagnostic type 1 Brugada ECG (Sp1), history of syncope likely caused by ventricular rugada syndrome (BrS), characterized by ST-segment elevation in the right precordial leads (type 1 Brugada electrocardiogram [ECG]) and a high incidence of ventricular fibrillation (VF) leading to sudden cardiac death (SCD) in young and otherwise healthy adults, has been reported since the initial description of the disease. 1 It is estimated to be responsible for at least 4% of all sudden deaths and at least 20% of sudden deaths in patients with structurally normal hearts. 2 Clearly, a method for the risk stratification of patients with BrS is required. A number of studies have noted a high B
Circulation. Arrhythmia and electrophysiology, 2015
I mplantable cardioverter defibrillator (ICD) is considered to be the main therapy for prevention of sudden cardiac death in patients with Brugada syndrome (BrS). However, there have been several reports suggesting a higher prevalence of complications, such as inappropriate shocks, lead failure, and device infection compared with the incidence of lethal ventricular arrhythmia. 1 BrS is mainly diagnosed ≈40 years of age in men, 2,3 and sudden death is likely to be eliminated after ICD implantation. Meanwhile, the clinical characteristics and prognosis of elderly patients with BrS are largely unknown, and there are no reports on the incidence of ventricular fibrillation (VF) or the evaluations of the necessity for ICD in elderly patients. Provided that BrS is caused by depolarization abnormality, 4 ventricular arrhythmias may conceivably increase with age. The purpose of this study was to investigate the incidence of appropriate shocks and ICD complications in patients with BrS during long-term follow-up after ICD implantation and to assess the risk and benefit of ICD implantation in elderly patients with BrS. Methods Study Population The study population consisted of 120 consecutive BrS patients who were admitted to National Cerebral and Cardiovascular Center, Suita, Japan, between 1992 and 2013, and had undergone ICD implantation (115 men; mean age, 46.6±12.2 years). All patients except 1 were index cases. None of all the patients had structural heart disease, including arrhythmogenic right ventricular cardiomyopathy, which was confirmed by noninvasive studies (physical examination, 12-lead ECG, 87-lead body surface ECG, exercise stress test, signal-averaged electrocardiography, and cardiac magnetic resonance imaging or computed tomography), and invasive studies consisting of coronary angiography including ergonovine/acetylcholine injection and right or left ventricular cineangiography. This study was approved by the Institutional Research Board of National Cerebral and Cardiovascular Center. Diagnosis of Brugada Syndrome BrS was diagnosed when a type 1 ST-segment elevation was observed either spontaneously or after intravenous administration of a sodium channel blocking agent in at least 1 right precordial lead (V 1 and V 2), which was placed in a standard or a superior position (up to the
2018
BACKGROUND Limited information exists about detailed clinical characteristics and management of the small subset of Brugada syndrome (BrS) patients who had an arrhythmic event (AE). OBJECTIVES To conduct the first nationwide survey focused on BrS patients with documented AE. METHODS Israeli electrophysiology units participated if they had treated BrS patients who had cardiac arrest (CA) (lethal/aborted; group 1) or experienced appropriate therapy for tachyarrhythmias after prophylactic implantable cardioverter defibrillator (ICD) implantation (group 2). RESULTS The cohort comprised 31 patients: 25 in group 1, 6 in group 2. Group 1: 96% male, mean CA age 38 years (range 13-84). Nine patients (36%) presented with arrhythmic storm and three had a lethal outcome; 17 (68%) had spontaneous type 1 Brugada electrocardiography (ECG). An electrophysiology study (EPS) was performed on 11 patients with inducible ventricular fibrillation (VF) in 10, which was prevented by quinidine in 9/10 patie...
Heart Rhythm, 2014
BACKGROUND All available studies that have addressed the issue of risk stratification in patients with type 1 Brugada electrocardiographic (ECG) pattern have considered a combined end point constituted by implantable cardioverter-defibrillator-recorded fast ventricular arrhythmias (ICD-FVA) and sudden death (SD) in patients without ICD. OBJECTIVE As ICD-FVA are only a surrogate of SD, we tried to focus on the prognostic value of classical risk factors by separating patients with ICD-FVA from those without ICD who suffered SD. METHODS We made a cumulative analysis of the largest available studies. Studies were selected in which the incidence of FVA and SD could be determined in patients with and without ICD separately. In addition, we tried to analyze the prognostic value of risk factors in patients with and without ICD separately. RESULTS A total of 2176 patients were recruited from 5 studies, about one-third of whom had an ICD and two-thirds did not. Event rates per 1000 patient-years of follow-up were 31.3 (25-39) and 6.5 (4-10) in patients with and without ICD, respectively (P o .001). When considering FVA in patients with ICD, each single risk factor (spontaneous type 1 ECG pattern, familial juvenile SD, and þEPS) displayed limited clinical value, mainly owing to its low specificity (21%-61%) and low positive predictive value (9%-15%). CONCLUSIONS In patients with type 1 Brugada ECG pattern, most arrhythmic events occur in patients with an ICD while SD is rare in patients without an ICD. While we have an acceptable ability to predict ICD-FVA, we have insufficient data to predict SD.
2018
Aims Data on predictors of time-to-first appropriate implantable cardioverter-defibrillator (ICD) therapy in patients with Brugada Syndrome (BrS) and prophylactically implanted ICD's are scarce. Methods and results SABRUS (Survey on Arrhythmic Events in BRUgada Syndrome) is an international survey on 678 BrS patients who experienced arrhythmic event (AE) including 252 patients in whom AE occurred after prophylactic ICD implantation. Analysis was performed on time-to-first appropriate ICD discharge regarding patients' characteristics. Multivariate logistic regression models were utilized to identify which parameters predicted time to arrhythmia ≤5 years. The median time-to-first appropriate ICD therapy was 24.8 ± 2.8 months. A shorter time was observed in patients from Asian ethnicity (P < 0.05), those with syncope (P = 0.001), and those with Class IIa indication for ICD (P = 0.001). A longer time was associated with a positive family history of sudden cardiac death (P <...
Circulation, 2006
Background-Brugada syndrome is an arrhythmogenic disease characterized by an increased risk of sudden cardiac death (SCD) by ventricular fibrillation. At present, an implantable cardioverter-defibrillator (ICD) is the recommended therapy in high-risk patients. This multicenter study reports the outcome of a large series of patients implanted with an ICD for Brugada syndrome. Methods and Results-All patients (nϭ220, 46Ϯ12 years, 183 male) with a type 1 Brugada ECG pattern implanted with an ICD in 14 centers between 1993 and 2005 were investigated. ICD indication was based on resuscitated SCD (18 patients, 8%), syncope (88 patients, 40%), or positive electrophysiological study in asymptomatic patients (99 patients, 45%). The remaining 15 patients received an ICD because of a family history of SCD or nonsustained ventricular arrhythmia. During a mean follow-up of 38Ϯ27 months, no patient died and 18 patients (8%) had appropriate device therapy (10Ϯ15 shocks/patient, 26Ϯ33 months after implantation). The complication rate was 28%, including inappropriate shocks, which occurred in 45 patients (20%, 4Ϯ3 shocks/patient, 21Ϯ20 months after implantation). The reasons for inappropriate therapy were lead failure (19 patients), T-wave oversensing (10 patients), sinus tachycardia (10 patients), and supraventricular tachycardia (9 patients). Among implantation parameters, high defibrillation threshold, high pacing threshold, and low R-wave amplitude occurred, respectively, in 12%, 27%, and 15% of cases. Conclusion-In this large Brugada syndrome population, a low incidence of arrhythmic events was found, with an annual event rate of 2.6% during a follow-up of Ͼ3 years, in addition to a significant risk of device-related complications (8.9%/year). Inappropriate shocks were 2.5 times more frequent than appropriate ones.
Heart rhythm, 2018
There is limited information on gender differences in patients with Brugada syndrome (BrS) who experienced arrhythmic events (AEs). To compare clinical, electrocardiographic (ECG), electrophysiologic (EP) and genetic characteristics between males and females in BrS-patients with their first AE. The multicenter Survey on AE in BrS (SABRUS) collected data on first AE in 678 BrS-patients including 619 (91.3%) males and 59 (8.7%) females aged 0.27 to 84 (mean 42.5±14.1) years at the time of AE. After excluding pediatric patients, females were older than males (49.5±14.4 vs. 43±12.7 years, respectively, P=0.001). Higher proportions of females were observed in the pediatric and elderly populations. In Asians, male/female ratio of AE was ≈9-fold higher compared to Caucasians. Spontaneous type 1 BrS-ECG was associated with earlier onset of AE in pediatric females. A similar prevalence (≈65%) of spontaneous type 1 BrS-ECG was present in males and females above age of 60 years. Females less f...
International Journal of Cardiology, 2010
Background: Brugada syndrome (BS) is associated with an increased risk of sudden cardiac death (SCD) caused by ventricular tachyarrhythmia. Thus, implantable cardioverter defibrillators (ICD) became the main therapeutic option in these patients. We aimed to investigate the prevalence of BS in the Eastern Alps as well as the benefit of ICD therapy in this collective. Methods: During physical examination before military service, 47,606 Austrian men were screened for Brugada ECG pattern. Furthermore, we followed 4491 patients with arrhythmia during the last two decades, of which 26 patients (20 male; age at diagnosis: 43.2 ± 11.6 years) revealed BS. Diagnosis was based on characteristic ECG either at rest (11 patients) or after provocation with Ajmaline (15 patients). Results: The nationwide screening revealed one individual with Brugada ECG (prevalence of 2.10/100,000 inhabitants). Prior to diagnosis of BS, syncope and SCD survival were observed in 7 and 4 patients, respectively; the remaining 15 patients were asymptomatic. ICD were implanted in 17 patients (15 male). Three asymptomatic patients received no ICD because no tachyarrhythmia was inducible on programmed stimulation. Six asymptomatic patients without family history of sudden death refused further evaluation. Mean ICD follow-up period was 57.0 ± 32.2 months. Two patients (11.7%) needed defibrillation therapy. Four patients (23.5%) received exclusively inappropriate shocks (three due to T-wave oversensing, one due to atrial fibrillation). Conclusions: Brugada syndrome has a low prevalence in the Eastern alpine region. Patients with BS benefit from ICD implantation, but less frequently than anticipated. The problem of inappropriate ICD discharges is still of major concern.
The Israel Medical Association journal : IMAJ, 2008
Many electrophysiologists recommend implantable cardioverter defibrillators for patients with Brugada syndrome who are cardiac arrest survivors or presumed at high risk of sudden death (patients with syncope or a familial history of sudden death or those with inducible ventricular fibrillation at electrophysiologic study). To assess the efficacy and complications of ICD therapy in patients with Brugada syndrome. The indications, efficacy and complications of ICD therapy in all patients with Brugada syndrome who underwent ICD implantation in 12 Israeli centers between 1994 and 2007 were analyzed. There were 59 patients (53 males, 89.8%) with a mean age of 44.1 years. At diagnosis 42 patients (71.2%) were symptomatic while 17 (28.8%) were asymptomatic. The indications for ICD implantation were: a history of cardiac arrest (n = 11, 18.6%), syncope (n = 31, 52.5%), inducible VF in asymptomatic patients (n = 14, 23.7%), and a family history of sudden death (n = 3, 0.5%). The overall indu...