Justo Juliá

Implantable Cardioverter-Defibrillator Therapy in Brugada Syndrome: A 20-Year Single-Center Experience

BACKGROUND Patients with Brugada syndrome and aborted sudden cardiac death or syncope have higher risks for ventricular arrhythmias (VAs) and should undergo implantable cardioverter-defibrillator (ICD) placement. Device-based management of asymptomatic patients is controversial. ICD therapy is associated with high rates of inappropriate shocks and device-related complications. OBJECTIVES The objective of this study was to investigate clinical features, management, and long-term follow-up of ICD therapy in patients with Brugada syndrome. METHODS Patients presenting with spontaneous or drug-induced Brugada type 1 electrocardiographic findings, who underwent ICD implantation and continuous follow-up at a single institution, were eligible for this study. RESULTS A total of 176 consecutive patients were included. During a mean follow-up period of 83.8 ± 57.3 months, spontaneous sustained VAs occurred in 30 patients (17%). Eight patients (4.5%) died. Appropriate ICD shocks occurred in 28 patients (15.9%), and 33 patients (18.7%) had inappropriate shocks. Electrical storm occurred in 4 subjects (2.3%). Twenty-eight patients (15.9%) experienced device-related complications. In multivariate Cox regression analysis, aborted sudden cardiac death and VA inducibility on electrophysiologic studies were independent predictors of appropriate shock occurrence. CONCLUSIONS ICD therapy was an effective strategy in Brugada syndrome, treating potentially lethal arrhythmias in 17% of patients during long-term follow-up. Appropriate shocks were significantly associated with the presence of aborted sudden cardiac death but also occurred in 13% of asymptomatic patients. Risk stratification by electrophysiologic study may identify asymptomatic patients at risk for arrhythmic events and could be helpful in investigating syncope not related to VAs. ICD placement is frequently associated with device-related complications, and rates of inappropriate shocks remain high regardless of careful device programming.

Prognostic Value of Programmed Electrical Stimulation in Brugada Syndrome 20 Years Experience

Background—The prognostic value of electrophysiological investigations in individuals with Brugada syndrome remains controversial. Different groups have published contradictory data. Long-term follow-up is needed to clarify this issue. Methods and Results—Patients presenting with spontaneous or drug-induced Brugada type I ECG and in whom programmed electric stimulation was performed at our institution were considered eligible for this study. A total of 403 consecutive patients (235 males, 58.2%; mean age, 43.2±16.2 years) were included. Ventricular arrhythmias during programmed electric stimulation were induced in 73 (18.1%) patients. After a mean follow-up time of 74.3±57.3 months (median 57.3), 25 arrhythmic events occurred (16 in the inducible group and 9 in the noninducible). Ventricular arrhythmias inducibility presented a hazard ratio for events of 8.3 (95% confidence interval, 3.6–19.4), P<0.01. Conclusions—Programmed ventricular stimulation of the heart is a good predictor of outcome in individuals with Brugada syndrome. It might be of special value to guide further management when performed in asymptomatic individuals. The overall accuracy of the test makes it a suitable screening tool to reassure noninducible asymptomatic individuals

Asymptomatic Brugada Syndrome: Clinical Characterization and Long Term Prognosis

Background—Among Brugada syndrome patients, asymptomatic individuals are considered to be at the lowest risk. Nevertheless, arrhythmic events and sudden cardiac death are not negligible. Literature focused on this specific group of patients is sparse. The purpose of this study is to investigate the clinical characteristics, management, and long-term prognosis of asymptomatic Brugada syndrome patients. Methods and Results—Patients presenting with spontaneous or drug-induced Brugada type I ECG and no symptoms at our institution were considered eligible. A total of 363 consecutive patients (200 men, 55.1%; mean age, 40.9±17.2 years; 41 [11.3%] with spontaneous type I ECG) were included. Electrophysiological study was performed in 321 (88.4%) patients, and ventricular arrhythmias were induced in 32 (10%) patients. An implantable cardioverter defibrillator was implanted in 61 (16.8%) patients. After a mean follow-up time of 73.2±58.9 months, 9 arrhythmic events occurred, accounting for an annual incidence rate of 0.5%. Event-free survival was 99.0% at 1 year, 96.2% at 5 years, and 95.4% at 10 and 15 years. Univariate analysis identified as risk factors: electrophysiological study inducibility (hazard ratio, 11.4; P<0.01), spontaneous type I (hazard ratio, 4.0; P=0.04), and previous sinus node dysfunction (hazard ratio, 8.0; 95% confidence interval, 1.0–63.9; P=0.05). At the multivariate analysis, only inducibility remained significant (hazard ratio, 9.1; P<0.01) Conclusions—Arrhythmic events in asymptomatic Brugada syndrome patients are not insignificant. Ventricular arrhythmia inducibility, spontaneous type I ECG, and presence of sinus node dysfunction might be considered as risk factors and used to drive long-term management.

Clinical characterisation and long-term prognosis of women with Brugada syndrome.

Objectives Brugada syndrome (BS) in women is considered an infrequent condition with a more favourable prognosis than in men. Nevertheless, arrhythmic events and sudden cardiac death (SCD) also occur in this population. Long-term follow-up data of this group are sparse. The purpose of the present study was to investigate the clinical characteristics and long-term prognosis of women with BS. Methods A consecutive cohort of 228 women presenting with spontaneous or drug-induced Brugada type I ECG at our institution were included and compared with 314 men with the same diagnosis. Results Mean age was 41.5±17.3 years. Clinical presentation was SCD in 6 (2.6%), syncope in 51 (22.4%) and the remaining 171 (75.0%) were asymptomatic. As compared with men, spontaneous type I ECG was less common (7.9% vs 23.2%, p<0.01) and less ventricular arrhythmias were induced during programmed electrical stimulation (5.5% vs 22.3%, p<0.01). An implantable cardioverter defibrillator (ICD) was implanted in 64 women (28.1%). During a mean follow-up of 73.2±56.2 months, seven patients developed arrhythmic events, constituting an event rate of 0.7% per year (as compared with 1.9% per year in men, p=0.02). Presentation as SCD or sinus node dysfunction (SND) was risk factor significantly associated with arrhythmic events (hazard risk (HR) 25.4 and 9.1). Conclusion BS is common in women, representing 42% of patients in our database. Clinical presentation is less severe than men, with more asymptomatic status and less spontaneous type I ECG and prognosis is more favourable, with an event rate of 0.7% year. However, women with SCD or previous SND are at higher risk of arrhythmic events.

Follow-up From Childhood to Adulthood of Individuals With Family History of Brugada Syndrome and Normal Electrocardiograms

Follow-up From Childhood to Adulthood of Individuals With Family History of Brugada Syndrome and Normal Electrocardiograms Brugada syndrome is an inherited primary arrhythmia syndrome characterized by coved-type ST-segment elevation in the right precordial leads without structural heart disease and is associated with increased risk of sudden death.1 The syndrome is genetically and clinically heterogeneous and can present within the first months of life, although more typically in the fourth or fifth decade. The diagnostic type 1 electrocardiogram (ECG) may manifest spontaneously, increasing with age.2 Sex hormones have been suggested as potential responsible factors.3 Ajmaline challenge is recommended to unmask the diagnostic ECG pattern in patients with suspected disease.4 Although screening of first-degree relatives is common, no evidence-based guidelines exist, particularly for children with normal ECGs. We investigated the clinical significance of repeat testing after puberty in asymptomatic children with a family history of Brugada syndrome who had an initial negative ajmaline test.

A score model to predict risk of events in patients with Brugada Syndrome

Aims Risk stratification in Brugada Syndrome (BS) remains challenging. Arrhythmic events can occur life-long and studies with long follow-ups are sparse. The aim of our study was to investigate long-term prognosis and risk stratification of BS patients. Methods and results A single centre consecutive cohort of 400 BS patients was included and analysed. Mean age was 41.1 years, 78 patients (19.5%) had a spontaneous type I electrocardiogram (ECG). Clinical presentation was aborted sudden cardiac death (SCD) in 20 patients (5.0%), syncope in 111 (27.8%) and asymptomatic in 269 (67.3%). Familial antecedents of SCD were found in 184 individuals (46.0%), in 31 (7.8%) occurred in first-degree relatives younger than 35 years. An implantable cardioverter defibrillator (ICD) was placed in 176 (44.0%). During a mean follow-up of 80.7 months, 34 arrhythmic events occurred (event rate: 1.4% year). Variables significantly associated to events were: presentation as aborted SCD (Hazard risk [HR] 20.0), syncope (HR 3.7), spontaneous type I (HR 2.7), male gender (HR 2.7), early SCD in first-degree relatives (HR 2.9), SND (HR 5.0), inducible VA (HR 4.7) and proband status (HR 2.1). A score including ECG pattern, early familial SCD antecedents, inducible electrophysiological study, presentation as syncope or as aborted SCD and SND had a predictive performance of 0.82. A score greater than 2 conferred a 5-year event probability of 9.2%. Conclusions BS patients remain at risk many years after diagnosis. Early SCD in first-degree relatives and SND are risk factors for arrhythmic events. A simple risk score might help in the stratification and management of BS patients.

P-wave indices as predictors of atrial fibrillation recurrence after pulmonary vein isolation in normal left atrial size.

Aims Prolonged P-wave duration and dispersion are universally accepted noninvasive markers for atrial electrical remodeling. Our aim was to analyze P-wave indices as predictors of atrial fibrillation recurrence after pulmonary vein isolation in patients with normal left atrial size. Methods From January 2008 to December 2011, 426 patients with drug-resistant symptomatic paroxysmal atrial fibrillation underwent pulmonary vein isolation as an index procedure by conventional radiofrequency or cryoballoon ablation in our center. Patients with left atrial dilatation, poor-quality electrocardiograms, atrial pacemaker stimulation, and those undergoing repeat procedures were excluded. A total of 201 patients were analyzed during a mean follow-up of 22 ± 16 months. Results Patients with prolonged P-wave duration had higher rates of atrial fibrillation recurrences compared with those without prolonged P-wave duration (49 vs. 14%; P < 0.001). Atrial fibrillation recurrence was significantly associated with prolonged P-wave duration (129 ± 13 vs. 119 ± 11 ms; P < 0.001) and P-wave dispersion (54 ± 12 vs. 42 ± 10 ms; P < 0.001) compared with those who remained in sinus rhythm. P-wave duration and dispersion were independently associated with atrial fibrillation recurrence (hazard ratio 1.045, 95% confidence interval 1.027–1.063, P < 0.001; and hazard ratio 1.049, 95% confidence interval 1.022–1.078, P < 0.001, respectively), after adjusting for left atrial size and age. Conclusion Prolonged P-wave duration and dispersion were found to be independently associated with higher recurrence rates of atrial fibrillation after pulmonary vein isolation in patients with normal left atrial dimension. Therefore, a prolongation of P-wave indices may help to identify those patients in whom electrical remodeling has already occurred and a more extensive ablation may be indicated.