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.

Permanent pacemaker implantation in octogenarians with unexplained syncope and positive electrophysiologic testing.

BACKGROUND: Syncope is a common problem in the elderly, and a permanent pacemaker is a therapeutic option when a bradycardic etiology is revealed. However, the benefit of pacing when no association of symptoms to bradycardia has been shown is not clear, especially in the elderly. OBJECTIVE: The aim of this study was to evaluate the effect of pacing on syncope-free mortality in patients aged 80 years or older with unexplained syncope and "positive" invasive electrophysiologic testing (EPT). METHODS: This was an observational study. A positive EPT for the purposes of this study was defined by at least 1 of the following: a corrected sinus node recovery time of >525 ms, a basic HV interval of >55 ms, detection of infra-Hisian block, or appearance of second-degree atrioventricular block on atrial decremental pacing at a paced cycle length of >400 ms. RESULTS: Among the 2435 screened patients, 228 eligible patients were identified, 145 of whom were implanted with a pacemaker. Kaplan-Meier analysis determined that time to event (syncope or death) was 50.1 months (95% confidence interval 45.4-54.8 months) with a pacemaker vs 37.8 months (95% confidence interval 31.3-44.4 months) without a pacemaker (log-rank test, P = .001). The 4-year time-dependent estimate of the rate of syncope was 12% vs 44% (P < .001) and that of any-cause death was 41% vs 56% (P = .023), respectively. The multivariable odds ratio was 0.25 (95% confidence interval 0.15-0.40) after adjustment for potential confounders. CONCLUSION: In patients with unexplained syncope and signs of sinus node dysfunction or impaired atrioventricular conduction on invasive EPT, pacemaker implantation was independently associated with longer syncope-free survival. Significant differences were also shown in the individual components of the primary outcome measure (syncope and death from any cause).

Differences in the yield of the implantable loop recorder between secondary and tertiary centers.

BACKGROUND: The implantable loop recorder (ILR) is a useful tool for diagnosis of syncope or palpitations. Its easy use and safety have extended its use to secondary hospitals (those without an Electrophysiology Lab). The aim of the study was to compare results between secondary and tertiary hospitals. METHODS: National prospective and multicenter registry of patients with an ILR inserted for clinical reasons. Data were collected in an online database. The follow-up ended when the first diagnostic clinical event occurred, or 1 year after implantation. Data were analyzed according to the center of reference; hospitals with Electrophysiology Lab were considered Tertiary Hospitals, while those hospitals without a lab were considered Secondary Hospitals. RESULTS: Seven hundred and forty-three patients (413 [55.6%] men; 65 ± 16 year-old): 655 (88.2%) from Tertiary Centers (TC) and 88 (11.8%) from Secondary Centers (SC). No differences in clinical characteristics between both groups were found. The electrophysiologic study and the tilt table test were conducted more frequently in Tertiary Centers. Follow-up was conducted for 680 (91.5%) patients: 91% in TC and 94% in SC. There was a higher rate of final diagnosis among SC patients (55.4% vs. 30.8%; p < 0.001). Tertiary Hospital patients showed a trend towards a higher rate of neurally mediated events (20% vs. 4%), while bradyarrhythmias were more frequent in SC (74% vs. 60%; p = 0.055). The rate of deaths and adverse events was similar in both populations. CONCLUSIONS: Patients with an ILR in SC and TC have differences in terms of the use of complementary tests, but not in clinical characteristics. There was a higher rate of diagnosis in Secondary Hospital patients.

Sudden death risk in syncope: the role of the implantable cardioverter defibrillator.

Syncope is generally benign but when it is due to an underlying cardiovascular condition, the prognosis can be guarded. Patients with syncope may be at risk of dying suddenly from a ventricular arrhythmia especially if the collapse is caused by a poorly-tolerated, self-terminating, ventricular tachycardia (VT). If a similar VT recurs, and persists, it could initiate cardiac arrest, leading to sudden cardiac death. However, distinguishing which patient with syncope may benefit most from implantable cardioverter defibrillator (ICD) therapy, which can stop life-threatening and poorly tolerated VT, thereby preventing sudden cardiac death, remains an ongoing challenge. Careful assessment of the patient's underlying cardiovascular conditions, scrupulous attention to historical detail to assess potential causes for syncope, and risk stratification based upon clinical characteristics and short and long-term risks can help. This review focuses on the sudden death risk in patients with syncope and explores the role of the ICD to treat ventricular arrhythmias, prevent symptoms, and prevent death.

Implantable cardioverter-defibrillator therapy improves long-term survival in patients with unexplained syncope, cardiomyopathy, and a negative electrophysiologic study.

OBJECTIVES: The purpose of this study was to evaluate the long-term outcomes of patients with unexplained syncope, ischemic or nonischemic cardiomyopathy, and a negative electrophysiologic study (EPS). BACKGROUND: EPS is frequently performed to evaluate syncope in patients with left ventricular dysfunction. Limited long-term data evaluating all-cause mortality in patients with no inducible arrhythmia or examining the potential benefits from implantable cardioverter-defibrillator (ICD) therapy are available. METHODS: We evaluated 102 consecutive patients with unexplained syncope, cardiomyopathy, and a negative EPS from September 1996 to December 2000. A blinded matched case-control analysis utilized 51 of these patients (19 treated with an ICD and 32 matched controls treated with conventional therapy). We compared primary endpoint of death and documented cardiac arrest of patients treated with ICD therapy to matched controls. RESULTS: Baseline characteristics were similar between groups. There were 14 primary events among the study population during a follow-up period of 44.3 +/- 20 months: 2 in the ICD group and 12 in the conventional therapy group. The hazard ratio for the risk of event in the ICD group compared with the conventional therapy group was 0.18 (95% confidence interval, 0.04-0.85; P = .04). Other comorbid conditions, including age, sex, ischemic etiology of heart failure, ejection fraction, and antiarrhythmic use, did not predict outcome. Appropriate ICD shocks occurred in 26% of patients at 2 years. CONCLUSIONS: This study suggests that empiric ICD therapy improves long-term outcomes in patients with unexplained syncope, ischemic or nonischemic cardiomyopathy, and negative EPS.

Role of programmed ventricular stimulation and implantable cardioverter defibrillators in patients with idiopathic dilated cardiomyopathy and syncope.

The aim of this study was to evaluate the role of programmed ventricular stimulation and ICDs in patients with idiopathic dilated cardiomyopathy and syncope. Between 1990 and 1998, 54 (mean age 67+/-11 years, 76% men) patients presented with idiopathic dilated cardiomyopathy and syncope. An electrophysiological study was done in 37 of the 54 patients: 10 had inducible sustained monomorphic ventricular tachycardia, 12 had conduction system disease or neurocardiogenic syncope, and 15 had a normal study. Overall, 17 patients received an ICD, 15 patients received a pacemaker, and 22 patients received no device. Nine of the 15 patients with a negative electrophysiological study eventually received an ICD: 3 because they were considered high risk and 6 because of recurrent syncope or presyncope. In the 17 patients who received an ICD, incidence of appropriate shocks at 1 and 3 years was 47% and 74%, respectively, in the inducible sustained monomorphic ventricular tachycardia group, and 40% and 40%, respectively, in the group without inducible sustained monomorphic ventricular tachycardia (P = 0.29, log-rank test). In conclusion, programmed ventricular stimulation is not useful in risk stratification of patients with idiopathic dilated cardiomyopathy and syncope and may delay necessary ICD implantation.