Drug-Induced Brugada Syndrome in Children Clinical Features, Device-Based Management, and Long-Term Follow-Up

ObjectivesThe goal of this study was to investigate the clinical features, management, and long-term follow-up of children with drug-induced Brugada syndrome (BS).BackgroundPatients with BS <12 years of age with a spontaneous type I electrocardiogram have a higher risk of arrhythmic events. Data on drug-induced BS in patients <12 years of age are lacking.MethodsAmong 505 patients with ajmaline-induced BS, subjects ≤12 years of age at the time of diagnosis were considered as children and eligible for this study.ResultsForty children (60% male; age 8 ± 2.8 years) were included. Twenty-four children (60%) had a family history of sudden death. Two (5%) had a previous episode of aborted sudden death, and 8 (20%) had syncope. Children experienced more frequent episodes of sinus node dysfunction (SND) compared with older subjects (7.5% vs. 1.5%; p = 0.04) and had a comparable incidence of atrial tachyarrhythmias. Children more frequently experienced episodes of ajmaline-induced sustained ventricular arrhythmias (VAs) compared with older patients (10.0% vs. 1.3%; p = 0.005). Twelve children (30%) received an implantable cardioverter-defibrillator (ICD). After a mean follow-up time of 83 ± 51 months, none of the children died suddenly. Spontaneous sustained VAs were documented in 1 child (2%). Among children with ICD, 1 (8%) experienced an appropriate shock, 4 (33%) had inappropriate ICD shocks, and 4 (33%) experienced device-related complications.ConclusionsDrug-induced BS is associated with atrial arrhythmias and SND. Children are at higher risk of ajmaline-induced VAs. The rate of device-related complications, leading to lead replacement or inappropriate shocks, is considerable and even higher than with appropriate interventions. Based on these findings, the optimal management of BS in childhood should remain individualized, taking into consideration the patient's clinical history and family's wishes

Out-of-hospital cardiac arrest due to idiopathic ventricular fibrillation in patients with normal electrocardiograms:results from a multicentre long-term registry

AIMS : To define the clinical characteristics and long-term clinical outcomes of a large cohort of patients with idiopathic ventricular fibrillation (IVF) and normal 12-lead electrocardiograms (ECGs). METHODS AND RESULTS: Patients with ventricular fibrillation as the presenting rhythm, normal baseline, and follow-up ECGs with no signs of cardiac channelopathy including early repolarization or atrioventricular conduction abnormalities, and without structural heart disease were included in a registry. A total of 245 patients (median age: 38 years; males 59%) were recruited from 25 centres. An implantable cardioverter-defibrillator (ICD) was implanted in 226 patients (92%), while 18 patients (8%) were treated with drug therapy only. Over a median follow-up of 63 months (interquartile range: 25-110 months), 12 patients died (5%); in four of them (1.6%) the lethal event was of cardiac origin. Patients treated with antiarrhythmic drugs only had a higher rate of cardiovascular death compared to patients who received an ICD (16% vs. 0.4%, P = 0.001). Fifty-two patients (21%) experienced an arrhythmic recurrence. Age ≤16 years at the time of the first ventricular arrhythmia was the only predictor of arrhythmic recurrence on multivariable analysis [hazard ratio (HR) 0.41, 95% confidence interval (CI) 0.18-0.92; P = 0.03]. CONCLUSION : Patients with IVF and persistently normal ECGs frequently have arrhythmic recurrences, but a good prognosis when treated with an ICD. Children are a category of IVF patients at higher risk of arrhythmic recurrences

PREVENT-VT trial: game changer of ischemic ventricular tachycardia therapy?

info:eu-repo/semantics/publishe

Atrial fibrillation outcomes: changing the paradigm.

LetterResearch Support, Non-U.S. Gov'tReviewinfo:eu-repo/semantics/publishe

Phrenic nerve paralysis during cryoballoon ablation for atrial fibrillation: a comparison between the first- and second-generation balloon.

Phrenic nerve palsy (PNP) is the most frequently observed complication during cryoballoon ablation (CB; Arctic Front, Medtronic, MN) occurring in roughly 7%-9% of the cases. The new second-generation cryoballoon ablation Arctic Front Advance (CB-A) (Arctic Front) has recently been launched in the market.Journal Articleinfo:eu-repo/semantics/publishe

Impact of transseptal puncture site on acute and mid-term outcomes during cryoballoon ablation: a comparison between anterior, medial and posterior transatrial access.

Cryoballoon ablation (CBA) (Arctic Front, Medtronic, USA) has proven very effective in achieving pulmonary vein isolation (PVI). Different transseptal (TS) puncture sites might influence CBA procedure. The aim of the present study was to analyze the influence of different TS puncture sites (anterior, medial and posterior) on the acute and midterm outcomes of CBA in a series of patients undergoing PVI for drug resistant AF.Journal Articleinfo:eu-repo/semantics/publishe

Long-Term Trends in Newly Diagnosed Brugada Syndrome: Implications for Risk Stratification

Background A proband of Brugada syndrome (BrS) is the first patient diagnosed in a family. There are no data regarding this specific, high-risk population. Objectives This study sought to investigate the Brugada probands diagnosed from 1986 through the next 28 years. Methods We included 447 probands belonging to families with a diagnostic type 1 electrocardiogram Brugada pattern. The database was divided into 2 periods: the first period identified patients who were part of the initial cohort that became the consensus document on BrS in 2002 (early group); the second period reflected patients first diagnosed from 2003 to January 2014 (latter group). Results There were 165 probands in the early group and 282 in the latter group. Aborted sudden death as the first manifestation of the disease occurred in 12.1% of the early group versus 4.6% of the latter group (p = 0.005). Inducibility during programmed electrical stimulation was achieved in 34.4% and 19.2% of patients, respectively (p < 0.001). A spontaneous type 1 electrocardiogram pattern at diagnosis was present in 50.3% early versus 26.2% latter patients (p = 0.0002). Early group patients had a higher probability of a recurrent arrhythmia during follow-up (19%) than those of the latter group (5%) (p = 0.007). The clinical suspicion and use of a sodium-channel blocker to unmask BrS has allowed earlier diagnoses in many patients. Conclusions Since being first described, the presentation of BrS has changed. There has been a decrease in aborted sudden cardiac death as the first manifestation of the disease among patients who were more recently diagnosed. These variations in initial presentation have important clinical consequences. In this setting, the value of inducibility to stratify individuals with BrS has changed.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Repeat procedure using radiofrequency energy for recurrence of atrial fibrillation after initial cryoballoon ablation: a 2-year follow-up.

The ideal energy source needed to perform 're-isolation' of the pulmonary veins (PVs) during a repeat procedure for recurrence of paroxysmal atrial fibrillation (AF) has not been established yet. In this study we analysed the outcome of repeat procedure using radiofrequency (RF) energy after initial cryoballoon (CB) ablation at 2-year follow-up.Journal Articleinfo:eu-repo/semantics/publishe