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Dataset results
40 results for “Brugada syndrome;”
GenLab: Unveiling the Genetic Landscape of Brugada Syndrome: Novel Biomarker Discovery for Precise Diagnosis
ClinicalTrials.gov study NCT06647927. IPD Sharing: Not stated. Countries: 1. Publications: 0.
The Brugada Syndrome: a Follow-up Study
ClinicalTrials.gov study NCT03485508. IPD Sharing: UNDECIDED. Countries: 1. Publications: 0.
Ripple Mapping for Epicardial Mapping of Brugada Syndrome
ClinicalTrials.gov study NCT03435393. IPD Sharing: NO. Countries: 3. Publications: 0.
UZ Brussel HRMC Registry of Brugada Syndrome
ClinicalTrials.gov study NCT05283759. IPD Sharing: NO. Countries: 1. Publications: 0.
Empagliflozin as a Potential Therapeutic Solution for Patients With Brugada Syndrome
ClinicalTrials.gov study NCT07146880. IPD Sharing: NO. Countries: 1. Publications: 0.
DAPERB 3,4-DiAminoPyridine and Electrophysiological Response in Brugada Syndrome
ClinicalTrials.gov study NCT00701077. IPD Sharing: Not stated. Countries: 1. Publications: 0.
Drug-induced Brugada Syndrome Research Database
ClinicalTrials.gov study NCT05048602. IPD Sharing: Not stated. Countries: 1. Publications: 0.
Feasibility of Improving Risk Stratification in Brugada Syndrome
ClinicalTrials.gov study NCT03992677. IPD Sharing: UNDECIDED. Countries: 1. Publications: 0.
The Conus Brugada Syndrome Study
ClinicalTrials.gov study NCT06653504. IPD Sharing: Not stated. Countries: 1. Publications: 0.
Hydroquinidine Versus Placebo in Patients With Brugada Syndrome
ClinicalTrials.gov study NCT00927732. IPD Sharing: Not stated. Countries: 1. Publications: 0.
AnalyST & Brugada Syndrome - Feasibility Study
ClinicalTrials.gov study NCT02052765. IPD Sharing: Not stated. Countries: 1. Publications: 0.
Empiric Quinidine for Asymptomatic Brugada Syndrome
ClinicalTrials.gov study NCT00789165. IPD Sharing: Not stated. Countries: 6. Publications: 0.
Rhythmic Risk of Type 1 Brugada Syndrome and Pulmonary Infundibulum Mapping
ClinicalTrials.gov study NCT03572881. IPD Sharing: NO. Countries: 1. Publications: 0.
A rare non-coding enhancer variant in SCN5A contributes to the high prevalence of Brugada syndrome in Thailand
GEO Series GSE264359. Homo sapiens. 8 samples. Type: Expression profiling by high throughput sequencing.
Data set from Monasky MM, Micaglio E, Vicedomini G, Locati ET, Ciconte G, Giannelli L, Giordano F, Crisà S, Vecchi M, Borrelli V, Ghiroldi A, D'Imperio S, Di Resta C, Benedetti S, Ferrari M, Santinelli V, Anastasia L, Pappone C. Comparable clinical characteristics in Brugada syndrome patients harboring SCN5A or novel SCN10A variants. Europace. 2019 Oct 1;21(10):1550-1558. doi: 10.1093/europace/euz186. PMID: 31292628.
<p>Data set from Monasky MM, Micaglio E, Vicedomini G, Locati ET, Ciconte G, Giannelli L, Giordano F, Crisà S, Vecchi M, Borrelli V, Ghiroldi A, D'Imperio S, Di Resta C, Benedetti S, Ferrari M, Santinelli V, Anastasia L, Pappone C. Comparable clinical characteristics in Brugada syndrome patients harboring SCN5A or novel SCN10A variants. Europace. 2019 Oct 1;21(10):1550-1558. doi: 10.1093/europace/euz186. PMID: 31292628.</p> <p> </p> <p>This is the abstract:</p> <p><strong>Aims: </strong>The Brugada syndrome (BrS) is an inherited disease associated with an increased risk of sudden cardiac death. Often, the genetic cause remains undetected. Perhaps due at least in part because the NaV1.8 protein is expressed more in both the central and peripheral nervous systems than in the heart, the SCN10A gene is not included in diagnostic arrhythmia/sudden death panels in the vast majority of cardiogenetics centres.</p> <p><strong>Methods and results: </strong>Clinical characteristics were assessed in patients harboring either SCN5A or novel SCN10A variants. Genetic testing was performed using Next Generation Sequencing on genomic DNA. Clinical characteristics, including the arrhythmogenic substrate, in BrS patients harboring novel SCN10A variants and SCN5A variants are comparable. Clinical characteristics, including gender, age, personal history of cardiac arrest/syncope, spontaneous BrS electrocardiogram pattern, family history of sudden death, and arrhythmic substrate are not significantly different between probands harboring SCN10A or SCN5A variants.</p> <p><strong>Conclusion: </strong>Future studies are warranted to further characterize the role of these specific SCN10A variants.</p> <p> </p>
Data set from Ciconte G, Santinelli V, Vicedomini G, Borrelli V, Monasky MM, Micaglio E, Giannelli L, Negro G, Giordano F, Mecarocci V, Mazza BC, Locati E, Anastasia L, Calovic Z, Pappone C. Non-invasive assessment of the arrhythmogenic substrate in Brugada syndrome using signal-averaged electrocardiogram: clinical implications from a prospective clinical trial. Europace. 2019 Dec 1;21(12):1900-1910. doi: 10.1093/europace/euz295. PMID: 31647530.
<p>Data set from Ciconte G, Santinelli V, Vicedomini G, Borrelli V, Monasky MM, Micaglio E, Giannelli L, Negro G, Giordano F, Mecarocci V, Mazza BC, Locati E, Anastasia L, Calovic Z, Pappone C. Non-invasive assessment of the arrhythmogenic substrate in Brugada syndrome using signal-averaged electrocardiogram: clinical implications from a prospective clinical trial. Europace. 2019 Dec 1;21(12):1900-1910. doi: 10.1093/europace/euz295. PMID: 31647530.</p> <p> </p> <p>This is the abstract:</p> <p><strong>Aims: </strong> Brugada syndrome (BrS) represents a major cause of sudden cardiac death in young individuals. The risk stratification to forecast future life-threatening events is still controversial. Non-invasive assessment of late potentials (LPs) has been proposed as a risk stratification tool. However, their nature in BrS is still undetermined. The purpose of this study is to assess the electrophysiological determinants of non-invasive LPs.</p> <p><strong>Methods and results: </strong> Two hundred and fifty consecutive patients with (Group 1, n = 96) and without (Group 2, n = 154) BrS-related symptoms were prospectively enrolled in the registry. Signal-averaged electrocardiogram (SAECG) was performed in all subjects before undergoing epicardial mapping. Group 1 patients exhibited larger arrhythmogenic substrates (AS; 5.8 ± 2.8 vs. 2.6 ± 2.1 cm2, P < 0.001) with more delayed potentials (220.4 ± 46.0 vs. 186.7 ± 42.3 ms, P < 0.001). Late potentials were present in 82/96 (85.4%) Group 1 and in 31/154 (20.1%) Group 2 individuals (P < 0.001). Patients exhibiting LPs had more frequently a spontaneous Type 1 pattern (30.1% vs. 10.9%, P < 0.001), SCN5A mutation (34.5% vs. 21.2%, P = 0.02), and exhibited a larger AS with longer potentials (5.8 ± 2.7 vs. 2.2 ± 1.7 cm2; 231.2 ± 37.3 vs. 213.8 ± 39.0 ms; P < 0.001, respectively). Arrhythmogenic substrate dimension was the strongest predictor of the presence of LPs (odds ratio 1.9; P < 0.001). An AS area of at least 3.5 cm2 identified patients with LPs (area under the curve 0.88, 95% confidence interval 0.843-0.931; P < 0.001) with a sensitivity of 86%, specificity 88%, positive predictive value 85%, and negative predictive value 89%.</p> <p><strong>Conclusion: </strong> The results of this study support the role of the epicardial AS as an electrophysiological determinant of non-invasive LPs, which may serve as a tool in the non-invasive assessment of the BrS substrate, as SAECG-LPs could be considered an expression of the abnormal epicardial electrical activity.</p> <p>ClinicalTrials.gov number (<a href="http://clinicaltrials.gov/show/NCT02641431">NCT02641431</a>; <a href="http://clinicaltrials.gov/show/NCT03106701">NCT03106701</a>).</p> <p> </p>
Data set from Pappone C, Mecarocci V, Manguso F, Ciconte G, Vicedomini G, Sturla F, Votta E, Mazza B, Pozzi P, Borrelli V, Anastasia L, Micaglio E, Locati E, Monasky MM, Lombardi M, Calovic Z, Santinelli V. New electromechanical substrate abnormalities in high-risk patients with Brugada syndrome. Heart Rhythm. 2020 Apr;17(4):637-645. doi: 10.1016/j.hrthm.2019.11.019. Epub 2019 Nov 19. PMID: 31756528.
<p>Data set from Pappone C, Mecarocci V, Manguso F, Ciconte G, Vicedomini G, Sturla F, Votta E, Mazza B, Pozzi P, Borrelli V, Anastasia L, Micaglio E, Locati E, Monasky MM, Lombardi M, Calovic Z, Santinelli V. New electromechanical substrate abnormalities in high-risk patients with Brugada syndrome. Heart Rhythm. 2020 Apr;17(4):637-645. doi: 10.1016/j.hrthm.2019.11.019. Epub 2019 Nov 19. PMID: 31756528.</p> <p> </p> <p>This is the abstract:</p> <p><strong>Background: </strong> The relationship between the typical electrocardiographic pattern and electromechanical abnormalities has never been systematically explored in Brugada syndrome (BrS).</p> <p><strong>Objectives: </strong> The aims of this study were to characterize the electromechanical substrate in patients with BrS and to evaluate the relationship between electrical and mechanical abnormalities.</p> <p><strong>Methods: </strong> We enrolled 50 consecutive high-risk patients with BrS (mean age 42 ± 7.2 years), with implantable cardioverter-defibrillator implantation for primary or secondary prevention of ventricular tachyarrhythmias (ventricular tachycardia/ventricular fibrillation [VT/VF]), undergoing substrate mapping and ablation. Patients underwent 3-dimensional (3D) echocardiography with 3D wall motion/deformation quantification and electroanatomic mapping before and after ajmaline administration (1 mg/kg in 5 minutes); 3D mechanical changes were compared with 50 age- and sex-matched controls. The effect of substrate ablation on electromechanical abnormalities was also assessed.</p> <p><strong>Results: </strong> In all patients, ajmaline administration induced Brugada type 1 pattern, with a significant increase in the electrical substrate (P < .001), particularly in patients with previous spontaneous VT/VF (P = .007). Induction of Brugada pattern was associated with lowering of right ventricular (RV) ejection fraction (P < .001) and worsening of 3D RV mechanical function (P < .001), particularly in the anterior free wall of the RV outflow tract, without changes in controls. RV electrical and mechanical abnormalities were highly correlated (r = 0.728, P < .001). By multivariate analysis, only the area of RV dysfunction was an independent predictor of spontaneous VT/VF (odds ratio 1.480; 95% confidence interval 1.159-1.889; P = .002). Substrate ablation abolished both BrS-electrocardiographic pattern and mechanical abnormalities, despite ajmaline rechallenge.</p> <p><strong>Conclusion: </strong> BrS is an electromechanical disease affecting the RV. The typical BrS pattern reflects an extensive RV arrhythmic substrate, driving consistent RV mechanical abnormalities. Substrate ablation abolished both Brugada pattern and mechanical abnormalities.</p> <p> </p>
Brugada syndrome genetics is associated with phenotype severity.
<p>Dataset from Ciconte G, Monasky MM, Santinelli V, Micaglio E, Vicedomini G, Anastasia L, Negro G, Borrelli V, Giannelli L, Santini F, de Innocentiis C, Rondine R, Locati ET, Bernardini A, Mazza BC, Mecarocci V, Ćalović Ž, Ghiroldi A, D'Imperio S, Benedetti S, Di Resta C, Rivolta I, Casari G, Petretto E, Pappone C. Brugada syndrome genetics is associated with phenotype severity. Eur Heart J. 2021 Mar 14;42(11):1082-1090. doi: 10.1093/eurheartj/ehaa942. PMID: 33221895; PMCID: PMC7955973.</p> <p><strong>Abstract</strong></p> <p><strong>Aims: </strong>Brugada syndrome (BrS) is associated with an increased risk of sudden cardiac death due to ventricular tachycardia/fibrillation (VT/VF) in young, otherwise healthy individuals. Despite SCN5A being the most commonly known mutated gene to date, the genotype-phenotype relationship is poorly understood and remains uncertain. This study aimed to elucidate the genotype-phenotype correlation in BrS.</p> <p><strong>Methods and results: </strong>Brugada syndrome probands deemed at high risk of future arrhythmic events underwent genetic testing and phenotype characterization by the means of epicardial arrhythmogenic substrate (AS) mapping, and were divided into two groups according to the presence or absence of SCN5A mutation. Two-hundred probands (160 males, 80%; mean age 42.6 ± 12.2 years) were included in this study. Patients harbouring SCN5A mutations exhibited a spontaneous type 1 pattern and experienced aborted cardiac arrest or spontaneous VT/VF more frequently than the other subjects. SCN5A-positive patients exhibited a larger epicardial AS area, more prolonged electrograms and more frequently observed non-invasive late potentials. The presence of an SCN5A mutation explained >26% of the variation in the epicardial AS area and was the strongest predictor of a large epicardial area.</p> <p><strong>Conclusion: </strong>In BrS, the genetic background is the main determinant for the extent of the electrophysiological abnormalities. SCN5A mutation carriers exhibit more pronounced epicardial electrical abnormalities and a more aggressive clinical presentation. These results contribute to the understanding of the genetic determinants of the BrS phenotypic expression and provide possible explanations for the varying degrees of disease expression.</p>
Brugada Syndrome: New Insights From Cardiac Magnetic Resonance and Electroanatomical Imaging
<p>Dataset from Pappone C, Santinelli V, Mecarocci V, Tondi L, Ciconte G, Manguso F, Sturla F, Vicedomini G, Micaglio E, Anastasia L, Pica S, Camporeale A, Lombardi M. Brugada Syndrome: New Insights From Cardiac Magnetic Resonance and Electroanatomical Imaging. Circ Arrhythm Electrophysiol. 2021 Nov;14(11):e010004. doi: 10.1161/CIRCEP.121.010004. Epub 2021 Oct 25. PMID: 34693720.</p> <p>Abstract</p> <p><strong>Background: </strong>Brugada syndrome (BrS) is considered a purely electrical disease with variable electrical substrates. Variable rates of mechanical abnormalities have been also reported. Whether exists a link between electrical and mechanical abnormalities has never been previously explored. This investigational physiopathological study aimed to determine the relationship between the substrate size/location, as exposed by ajmaline provocation, and the severity of mechanical abnormalities, as assessed by cardiac magnetic resonance in patients with BrS.</p> <p><strong>Methods: </strong>Twenty-four consecutive high-risk patients with BrS (mean age, 38±11 years, 17 males), presenting with malignant syncope and documented polymorphic ventricular tachycardia/ventricular fibrillation, and candidate to implantable cardioverter defibrillator implantation, underwent cardiac magnetic resonance and electroanatomic maps. During each examination, ajmaline test (1 mg/kg over 5 minutes) was performed. Cardiac magnetic resonance findings were compared with 24 age, sex, and body surface area-matched controls. In patients with BrS, the correlation between the electrical substrate extent and right ventricular regional mechanical abnormalities before/after ajmaline challenge was analyzed.</p> <p><strong>Results: </strong>After ajmaline, patients with BrS showed a reduction of right ventricular (RV) ejection fraction (<em>P</em><0.001), associated with decreased transversal displacement (U, <em>P</em><0.001) and longitudinal strain (ε, <em>P</em><0.001) localized at RV outflow tract. In patients with BrS significant preajmaline/postajmaline changes of transversal displacement (ΔU, <em>P</em><0.001) and longitudinal strain (Δε, <em>P</em><0.001) were found. In the control group, no mechanical changes were observed after ajmaline. The electrical substrate consistently increased after ajmaline from 1.7±2.8 cm<sup>2</sup> to 14.2±7.3 cm<sup>2</sup> (<em>P</em><0.001), extending from the RV outflow tract to the neighboring segments of the RV anterior wall. Postajmaline RV ejection fraction inversely correlated with postajmaline substrate extent (<em>r</em>=-0.830, <em>P</em><0.001). In patients with BrS and normal controls, cardiac magnetic resonance detected neither myocardial fibrosis nor RV outflow tract morphological abnormalities.</p> <p><strong>Conclusions: </strong>BrS is a dynamic RV electromechanical disease, where functional abnormalities correlate with the maximal extent of the substrate size.</p>
Alterations of the Sialylation Machinery in Brugada Syndrome
<p>Ghiroldi A, Ciconte G, Creo P, Tarantino A, Melgari D, D'Imperio S, Piccoli M, Cirillo F, Micaglio E, Monasky MM, Frosio A, Locati ET, Vicedomini G, Rivolta I, Pappone C, Anastasia L. Alterations of the Sialylation Machinery in Brugada Syndrome. Int J Mol Sci. 2022 Oct 29;23(21):13154. doi: 10.3390/ijms232113154. PMID: 36361941; PMCID: PMC9655504.</p> <p>Brugada Syndrome (BrS) is an inherited arrhythmogenic disorder with an increased risk of sudden cardiac death. Recent evidence suggests that BrS should be considered as an oligogenic or polygenic condition. Mutations in genes associated with BrS are found in about one-third of patients and they mainly disrupt the cardiac sodium channel NaV1.5, which is considered the main cause of the disease. However, voltage-gated channel's activity could be impacted by post-translational modifications such as sialylation, but their role in BrS remains unknown. Thus, we analyzed high risk BrS patients (<em>n</em> = 42) and healthy controls (<em>n</em> = 42) to assess an involvement of sialylation in BrS. Significant alterations in gene expression and protein sialylation were detected in Peripheral Blood Mononuclear Cells (PBMCs) from BrS patients. These changes were significantly associated with the phenotypic expression of the disease, as the size of the arrhythmogenic substrate and the duration of epicardial electrical abnormalities. Moreover, protein desialylation caused a reduction in the sodium current in an in vitro NaV1.5-overexpressing model. Dysregulation of the sialylation machinery provides definitive evidence that BrS affects extracardiac tissues, suggesting an underlying cause of the disease. Moreover, detection of these changes at the systemic level and their correlation with the clinical phenotype hint at the existence of a biomarker signature for BrS.</p>
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