P5027Prognosis and evaluation of the risk markers of arrhythmia in a large population of Brugada syndrome patients

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V Probst ◽  
S Anys ◽  
F Sacher ◽  
J Briand ◽  
B Guyomarch ◽  
...  
Keyword(s):  
Brugada Syndrome ◽  
Ventricular Arrhythmias ◽  
Clinical Status ◽  
Large Population ◽  
Fragmented Qrs ◽  
Increased Risk ◽  
Inherited Arrhythmia ◽  
St Elevation

Abstract Introduction Brugada syndrome (BrS) is an inherited arrhythmia syndrome with an increased risk of sudden cardiac death (SCD) despite a structurally normal heart. Many parameters have been suggested to be associated with the risk of ventricular arrhythmias, but only previous symptoms and spontaneous ECG pattern have been consistently associated with the risk of ventricular arrhythmia occurrence. Objective The aim of this study was to evaluate the association of these parameters with arrhythmic events in the largest cohort of BrS patients ever described. Methods Consecutive patients affected with BrS were recruited in a multicentric prospective registry in France (15 centers) between 1994 and 2016. Data were prospectively collected with an average follow-up of 6.5±4.7 years. ECGs were reviewed by 2 physicians blinded to clinical status. Results In this study, we enrolled a total of 1613 patients (mean age 45±15 years; 1119 males, 69%). At baseline, 462 patients (29%) were symptomatic (51 (3%) aborted SCD, 257 (16%) syncope). A spontaneous type 1 ECG pattern was present in 505 patients (31%). Implantable cardiac defibrillator was implanted in 477 patients (30%). During the follow-up, 91 patients (6%) underwent arrhythmic events (16 SCD (10%), 48 appropriate ICD therapy (3%) and 27 ventricular arrhythmias (2%). Thirty-six patients (2%) died of non-arrhythmic causes. Mean age at the first event was 44±15 years. In our cohort, event predictors were SCD (HR: 18.3; 95% CI: 11.2–29.8; p<0.0001), syncope (HR: 2.9; 95% CI: 1.8–4.9; p<0.0001), age >60 years (HR: 0.11; 95% CI: 0.032–0.377; p=0,0004), gender (HR: 2.96; 95% CI: 1.6–5.4; p=0.0005), spontaneous type 1 (HR: 2.14; 95% CI: 1.42–3.23; p=0.0003), type 1 ST elevation in peripheral ECG lead (HR: 3.6; 95% CI: 1.9–7.1; p=0,0001), fragmented QRS (HR: 3.37; 95% CI: 1.37–8.32; p=0,008), AvR sign (HR: 2.2; 95% CI: 1.4–3.8; p=0,0007), QRS >120ms in D2 lead (HR: 2.2; 95% CI: 1.4–3.6; p=0,001) and QRS >90ms in V6 (HR: 2.1; 95% CI: 1.3–3.3; p=0,001). All the others parameters including early repolarization pattern (ERP) and EPS were not predictor of events. Conclusion In the largest cohort of BrS patients ever described, we confirmed that symptoms, age, gender, spontaneous type 1, type 1 ST elevation in peripheral ECG lead, fragmented QRS, AvR sign, QRS >120ms in D2 and QRS >90ms in V6 are associated with arrhythmic events whereas ERP and EPS were not.

P6585Number of ECG leads and prognosis of spontaneous type 1 Brugada syndrome

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V Probst ◽  
M Arnaud ◽  
N Behar ◽  
P Mabo ◽  
B Guyomarch ◽  
...  
Keyword(s):  
Brugada Syndrome ◽  
Ventricular Arrhythmias ◽  
Icd Therapy ◽  
St Elevation ◽  
Group 3 ◽  
Group 2 ◽  
Ecg Leads ◽  
Group 1

Abstract Introduction Brugada syndrome (BrS) is an inherited arrhythmia syndrome with an increased risk of sudden cardiac death (SCD). The recent single lead-based diagnosis of Brugada syndrome recommended criterion may lead to overdiagnosis of Brugada syndrome and overestimation of the risk of SCD. Objective We aim to investigate the value of a single lead diagnosis in spontaneous type 1 ECG Brugada patient and to investigate the association between the number of ECG leads with a spontaneous type 1 ST elevation and the arrhythmic risk. Methods Consecutive patients affected with BrS were recruited in a multicentric prospective registry in France (15 centers) between 1994 and 2016. A total of 1613 patients affected by the Brugada syndrome were enrolled. For this specific study, only patient with a spontaneous type 1 BrS were enrolled (n=505). Data were prospectively collected with an average follow-up of 6.5±4.7 years. ECGs were reviewed by 2 physicians blinded to clinical status. Type 1 ST elevation was defined by ≥2 mm J-point elevation with coved ST segment and negative T wave. Results A total of 505 patients with a spontaneous type 1 BrS (mean age 46±15 years, 398 males, 79%) were enrolled. 117 patients (23%) were symptomatic at baseline (32 (6%) aborted SCD, 85 (17%) syncope). Implantable cardiac defibrillator (ICD) was implanted in 191 patients (38%). Brugada ECG pattern was found in 1 lead in 250 patients (50%, group 1), in 2 leads in 227 patients (45%, group 2) and in 3 leads in 28 patients (5%, group 3). Groups were comparable in term of clinical presentation except for group 3 who presented more frequently an early repolarization pattern (n=19 (8%) in group 1, n=15 in group 2 (6%) and n=7 (25%) in group 3, p=0.02) and more frequently QRS fragmentation (n=6 (2%) in group 1, n=3 in group 2 (1%) and n=3 (11%) in group 3, p=0.03). During follow-up, 46 (9%) patients presented an arrhythmic event: 22 (9%) in group 1 (4 SCD, 14 appropriate ICD therapy, 4 ventricular arrhythmias), 22 (10%) in group 2 (6 SCD, 11 appropriate ICD therapy, 5 ventricular arrhythmias) and 2 (7%) in group 3 (1 SCD, 1 appropriate ICD therapy). Patients with type 1 BrS pattern in 2 or 3 ECG leads had not a significantly higher rate of arrhythmic events than patients with type 1 BrS pattern in only 1 ECG lead (HR: 1.1; 95% CI: 0.6–1.9 for group 2 and HR: 0.7; 95% CI: 0.2–3 for group 2; p=0,087). Conclusion In the largest cohort of BrS patients ever described, the prognosis of Brugada syndrome with a spontaneous ECG pattern does not appear to be affected by the number of leads required for diagnostic.

scholarly journals Automated Electrocardiogram Analysis Identifies Novel Predictors of Ventricular Arrhythmias in Brugada Syndrome

2021 ◽  
Vol 7 ◽  
Author(s):  
Gary Tse ◽  
Sharen Lee ◽  
Andrew Li ◽  
Dong Chang ◽  
Guangping Li ◽  
...  
Keyword(s):  
Primary Endpoint ◽  
Brugada Syndrome ◽  
Ventricular Arrhythmias ◽  
Wave Amplitude ◽  
Cox Regression ◽  
S Wave ◽  
Wave Duration ◽  
Increased Risk ◽  
Tertiary Center

Background: Patients suffering from Brugada syndrome (BrS) are at an increased risk of life-threatening ventricular arrhythmias. Whilst electrocardiographic (ECG) variables have been used for risk stratification with varying degrees of success, automated measurements have not been tested for their ability to predict adverse outcomes in BrS.Methods: BrS patients presenting in a single tertiary center between 2000 and 2018 were analyzed retrospectively. ECG variables on vector magnitude, axis, amplitude and duration from all 12 leads were determined. The primary endpoint was spontaneous ventricular tachycardia/ventricular fibrillation (VT/VF) on follow-up.Results: This study included 83 patients [93% male, median presenting age: 56 (41–66) years old, 45% type 1 pattern] with 12 developing the primary endpoint (median follow-up: 75 (Q1–Q3: 26–114 months). Cox regression showed that QRS frontal axis &gt; 70.0 degrees, QRS horizontal axis &gt; 57.5 degrees, R-wave amplitude (lead I) &lt;0.67 mV, R-wave duration (lead III) &gt; 50.0 ms, S-wave amplitude (lead I) &lt; −0.144 mV, S-wave duration (lead aVL) &gt; 35.5 ms, QRS duration (lead V3) &gt; 96.5 ms, QRS area in lead I &lt; 0.75 Ashman units, ST slope (lead I) &gt; 31.5 deg, T-wave area (lead V1) &lt; −3.05 Ashman units and PR interval (lead V2) &gt; 157 ms were significant predictors. A weighted score based on dichotomized values provided good predictive performance (hazard ratio: 1.59, 95% confidence interval: 1.27–2.00, P-value&lt;0.0001, area under the curve: 0.84).Conclusions: Automated ECG analysis revealed novel risk markers in BrS. These markers should be validated in larger prospective studies.

scholarly journals 429 Incidence and management of adverse events among COVID-19 vaccination receipts with Brugada syndrome. Results from a multicentre-prospective European registry

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Rosa Cetera ◽  
Francesco Santoro ◽  
Domenico Gianfrancesco ◽  
Girolamo D’Arienzo ◽  
Francesco Bartolomucci ◽  
...  
Keyword(s):  
Body Temperature ◽  
Adverse Events ◽  
Brugada Syndrome ◽  
Inherited Disease ◽  
Increased Risk ◽  
Life Threatening ◽  
Inflammatory Drugs ◽  
Type 3

Abstract Aims Patients with Brugada syndrome have an increased risk of life-threatening arrhythmias when experience fever. Therefore, a prompt treatment of non-steroidal anti-inflammatory drugs (NSAI) is suggested in these patients. COVID-19 vaccination can be associated with fever in about 20% of patients. The aim of the study is to evaluate the incidence and management of adverse events within 48 h from COVID-19 vaccination among Brugada patients. Methods and results Eighty patients were enrolled from a prospective registry involving four European hospitals with a dedicated inherited disease ambulatory. Cardiological follow-up was performed within one month from vaccination. Mean age was 47 ± 17 years, 80% (num = 63) were male. Prevalence of Brugada types 1, 2, and 3 was as follows: 25% type 1, 39% type 2, and 24% type 3. Twenty-six percent of patients had an implantable cardioverter defibrillator (ICD). Within in 48 h from vaccination, 35% (num = 28) of patients experienced joint paint, 19% (num = 15) fever and 4% (num = 3) chest pain. In 8 out of 15 fever episodes body temperature was ≥38 degrees and treated with NSAI drugs. No patients had syncope or fatigue episodes and no arrhythmic episodes were recorded in patients with ICD. Conclusions About 20% of patients experienced fever after Covid-19 vaccination but no patient experienced life-threatening arrhythmias. Careful evaluation of body temperature and administration of NSAI in case of temperature higher than 38 is suggested.

scholarly journals A case report of a young patient with both Brugada and long QT3 syndrome: between the hammer and the anvil

2021 ◽  
Vol 5 (3) ◽  
Author(s):  
Ala Abu Dogoshh ◽  
Yuval Konstantino ◽  
Moti Haim
Keyword(s):  
Ventricular Arrhythmias ◽  
Beta Blockers ◽  
Channel Blockers ◽  
Loss Of Function ◽  
Case Summary ◽  
Increased Risk ◽  
Electrocardiogram Ecg ◽  
Scn5a Gene

Abstract Background Brugada syndrome (BrS) is an inherited disorder associated with increased risk of ventricular arrhythmias and sudden cardiac death. The most common genetic alteration is a loss of function mutation of SCN5A gene. Several mutations in SCN5A gene were found to be associated with an overlap phenotype of both BrS and long QT3 (LQT3) syndrome. Case summary We report of a 29-year-old man with familial LQT3 syndrome that was diagnosed at age 6 during evaluation of syncope. He has been treated for several years with Flecainide. Now presented with recurrent episodes of syncope. Electrocardiogram (ECG) upon admission was notable for Brugada type 1 pattern that was attenuated after Flecainide was discontinued. Genetic analysis revealed SCN5A 1790D&gt;G mutation that is associated with overlap of LQT3 and BrS. Due to recurrent syncope and difficult management of both LQT3 and BrS, an implantable cardioverter-defibrillator was implanted together with beta-blockers treatment. The patient was discharged home with no evidence of Brugada type 1 pattern on his ECG. He had no further syncope or arrhythmias during 6 months of follow-up. Discussion There are few reports describing the phenotypic overlap between LQT3 and BrS. Despite the confirmed genetic link between both syndromes, their management strategy is controversial. In particularly, the treatment with sodium channel blockers for LQT3 syndrome may increase the risk for arrhythmias in patients with coexisting BrS. The present case demonstrates the link between LQT3 and BrS and the difficult dilemma in the management of these patients.

scholarly journals Mechanism of ST Elevation and Ventricular Arrhythmias in an Experimental Brugada Syndrome Model

Circulation ◽  
2004 ◽  
Vol 109 (1) ◽  
pp. 125-131 ◽  
Author(s):  
Masaomi Kimura ◽  
Takao Kobayashi ◽  
Shingen Owada ◽  
Keiichi Ashikaga ◽  
Takumi Higuma ◽  
...  
Keyword(s):  
Brugada Syndrome ◽  
Ventricular Arrhythmias ◽  
St Elevation

scholarly journals Incidental Findings of Brugada Syndrome Type-1 and Epsilon-Like Pattern in Otherwise Healthy Man: a Case Report

2022 ◽  
Author(s):  
Brian Mendel ◽  
Valerie Dirjayanto ◽  
Radityo Prakoso ◽  
Sisca Siagian
Keyword(s):  
Brugada Syndrome ◽  
Unknown Origin ◽  
Initial Assessment ◽  
Structural Abnormality ◽  
Bundle Branch Block ◽  
Case Presentation ◽  
Electrophysiology Study ◽  
Esc Guidelines

Abstract Background: Brugada Syndrome (BrS) and arrhythmogenic right ventricle dysplasia (ARVD) are rare cardiomyopathies predisposing to sudden cardiac death (SCD). Comprehending the electrocardiographic features of these cardiomyopathies are crucial especially in emergency settings.Case presentation: A 25-year old medical student presented with no complaints, but had episodes of syncope, chest pain, and palpitations of unknown origin 10 years ago. The initial assessment showed stable hemodynamics. During examination, the ECG demonstrated incomplete right bundle branch block, Brugada-type 1 pattern, with signs of Epsilon wave. The following year, assessment of the ECG was repeated and findings were found suggestive of Brugada syndrome, although his echocardiography showed no structural abnormality. According to ESC guidelines, asymptomatic Brugada patients should undergo electrophysiology study.Conclusion: Careful follow-up with electrophysiology study is recommended for this patient in order to identify the likelihood of true Brugada and suitability for radiofrequency ablation or implantation of implantable cardioverter defibrillator (ICD).

scholarly journals Blood Neutrophil Counts are Associated with Exacerbation Frequency and Mortality in COPD

2020 ◽  
Author(s):  
Mike Lonergan ◽  
Alison J Dicker ◽  
Megan L Crichton ◽  
Holly R Keir ◽  
Melissa K. Van Dyke ◽  
...  
Keyword(s):  
Large Population ◽  
Real Life ◽  
Population Based ◽  
Blood Eosinophil ◽  
Blood Neutrophil ◽  
Disease Heterogeneity ◽  
Disease Information ◽  
Increased Risk ◽  
Eosinophil Counts

Abstract Background Identifying patients with COPD at increased risk of poor outcomes is challenging due to disease heterogeneity. Potential biomarkers need to be readily available in real-life clinical practice. Blood eosinophil counts are widely studied but few studies have examined the prognostic value of blood neutrophil counts (BNC). Methods In a large population-based COPD registry in the East of Scotland (TARDIS: Tayside Allergic and Respiratory Disease Information System), BNC were compared to measures of disease severity and mortality over up to 15 years follow-up. Potential mechanisms of disease modification by BNC were explored in a nested microbiome substudy. Results 178120 neutrophil counts were obtained from 7220 people (mean follow up 9 years) during stable disease periods. Median BNC was 5200cells/µL (IQR 4000-7000cells/µL). Mortality rates among those 34% with elevated BNCs (defined as 6000-15000cells/µL) at the study start were 80% higher (14.0/100 person years v 7.8/100py, P<0.001) than those with BNC in the normal range (2000-6000cells/µL). People with elevated BNC were more likely to be classified as GOLD D (46% v 33% P<0.001), have more exacerbations (mean 2.3 v 1.3/year, P<0.001), and were more likely to have severe exacerbations (13% vs. 5%, P<0.001) in the following year. Eosinophil counts were much less predictive of these outcomes. In a sub-cohort (N=276), patients with elevated BNC had increased relative abundance of Proteobacteria and reduced microbiome diversity. Conclusion High BNC may provide a useful indicator of risk of exacerbations and mortality in COPD patients.

scholarly journals SURVIVAL ADVANTAGE OF APOE2

2019 ◽  
Vol 3 (Supplement_1) ◽  
pp. S621-S621
Author(s):  
Sudha Seshadri
Keyword(s):  
Lower Risk ◽  
Large Population ◽  
Population Based ◽  
European Ancestry ◽  
Aggregated Data ◽  
Risk Of Death ◽  
Apoe Ε4 ◽  
Increased Risk ◽  
Ε4 Allele

Abstract Apolipoprotein E is a glycoprotein mediator and regulator of lipid transport and uptake. The APOE-ε4 allele has been associated with higher risk of Alzheimer’s disease and of mortality, but the effect of the less prevalent APOE-ε2 on survival remains elusive. We aggregated data of 38,537 individuals of European ancestry (mean age 65.5 years; 55.6% women) from six large population-based cohorts to determine the association of APOE-ε2, with survival in the general population. During a mean follow-up of 11.7 years, 17,021 individuals died. Compared with homozygous APOE-ε3 carriers, APOE-ε2 carriers were at lower risk of death (hazard ratio,95% confidence interval: 0.94,0.90-0.99; P=1.1*10-2), whereas APOE-ε4 carriers were at increased risk (HR 1.17,1.12-1.21; P=2.8*10-16). Risk was lowest for homozygous APOE-ε2 (HR 0.89,0.74-1.08), and highest for homozygous APOE-ε4 (HR 1.52,1.37-1.70). Results did not differ by sex. The association was unaltered after adjustment for baseline LDL or cardiovascular disease. Larger, multiethnic collaborations are ongoing.

scholarly journals Periaortic Abscess following DeBakey Type-1 Aortic Dissection Repair with Dacron Graft—Early Diagnosis and Management

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Rishi Raj ◽  
Dileep Unnikrishnan ◽  
Aasems Jacob ◽  
Kumar Ashish ◽  
Amulya Prakash ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Aortic Dissection ◽  
Fluid Collection ◽  
Dacron Graft ◽  
Surgical Drainage ◽  
Aortic Graft ◽  
Diagnosis And Management ◽  
Increased Risk

A 71-year-old male with history of DeBakey type-1 aortic dissection and repair with dacron graft three months prior to presentation was brought to the emergency room with complaints of high-grade fevers, chills, and shortness of breath. Chest X-ray revealed right lower lobe infiltrates and widened superior mediastinum. A follow-up CT chest with contrast showed fluid collection around the aortic graft. He was started on intravenous broad-spectrum antibiotics, and a TEE was done for further evaluation of periaortic fluid collection which showed findings to suggest periaortic abscess. The patient underwent surgical drainage of the abscess and was found to have an abscess around the surgical aortic graft which was drained followed by two weeks of antibiotic treatment. The patient was discharged to a rehabilitation facility and remained asymptomatic at three-month follow-up appointment. Type-1 aortic dissection is a medical emergency requiring acute surgical intervention, and despite significant advancements in diagnosis and management, the immediate and long-term complications remain high leading to increased risk of mortality. Our patient developed spontaneous periaortic abscess three months postoperatively requiring intensive antibiotic therapy along with surgical drainage. Our case emphasizes the importance of early diagnosis and management of late complications of periaortic abscess in patients with aortic dissection repair.

scholarly journals Acute Cardiogenic Shock Induced by Infusional 5-Fluorouracil

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Jeffrey Sulpher ◽  
Franco Dattilo ◽  
Susan Dent ◽  
Michele Turek ◽  
M. Neil Reaume ◽  
...  
Keyword(s):  
Wall Motion ◽  
Clinical Status ◽  
Metastatic Carcinoma ◽  
Prior History ◽  
History Of ◽  
St Elevation ◽  
Wall Motion Abnormalities ◽  
Carcinoma Of The Bladder ◽  
To Receive

A 49-year-old patient with metastatic carcinoma of the bladder and no prior history of heart disease presented with diffuse ST elevation, elevated troponins, and biventricular dysfunction requiring intensive care unit admission and inotropic support after receiving her first course of infusional 5-fluorouracil (5-FU). Over the course of several days, the patient’s cardiac function and clinical status returned to baseline. A follow-up echocardiogram performed 5 days after initial presentation revealed an ejection fraction of 59 percent, with no evidence of wall motion abnormalities. Subsequent 5-FU chemotherapy was discontinued, and the patient went on to receive second-line chemotherapy.

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