scholarly journals Fibrosis and Conduction Abnormalities as Basis for Overlap of Brugada Syndrome and Early Repolarization Syndrome

2021 ◽  
Vol 22 (4) ◽  
pp. 1570
Author(s):  
Bastiaan J. Boukens ◽  
Mark Potse ◽  
Ruben Coronel
Keyword(s):  
Brugada Syndrome ◽  
Heart Diseases ◽  
Imaging Techniques ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Early Repolarization ◽  
Structural Abnormalities ◽  
Structural Heart Diseases ◽  
The Right ◽  
Early Repolarization Syndrome

Brugada syndrome and early repolarization syndrome are both classified as J-wave syndromes, with a similar mechanism of arrhythmogenesis and with the same basis for genesis of the characteristic electrocardiographic features. The Brugada syndrome is now considered a conduction disorder based on subtle structural abnormalities in the right ventricular outflow tract. Recent evidence suggests structural substrate in patients with the early repolarization syndrome as well. We propose a unifying mechanism based on these structural abnormalities explaining both arrhythmogenesis and the electrocardiographic changes. In addition, we speculate that, with increasing technical advances in imaging techniques and their spatial resolution, these syndromes will be reclassified as structural heart diseases or cardiomyopathies.

scholarly journals Left Axis Deviation in Brugada Syndrome: Vectorcardiographic Evaluation during Ajmaline Provocation Testing Reveals Additional Depolarization Abnormalities

2021 ◽  
Vol 22 (2) ◽  
pp. 484
Author(s):  
Martijn H. van der Ree ◽  
Jeroen Vendrik ◽  
Jan A. Kors ◽  
Ahmad S. Amin ◽  
Arthur A. M. Wilde ◽  
...  
Keyword(s):  
Sodium Channel ◽  
Brugada Syndrome ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Conduction Delay ◽  
Channel Blockers ◽  
Axis Deviation ◽  
Provocation Testing ◽  
The Right

Patients with Brugada syndrome (BrS) can show a leftward deviation of the frontal QRS-axis upon provocation with sodium channel blockers. The cause of this axis change is unclear. In this study, we aimed to determine (1) the prevalence of this left axis deviation and (2) to evaluate its cause, using the insights that could be derived from vectorcardiograms. Hence, from a large cohort of patients who underwent ajmaline provocation testing (n = 1430), we selected patients in whom a type-1 BrS-ECG was evoked (n = 345). Depolarization and repolarization parameters were analyzed for reconstructed vectorcardiograms and were compared between patients with and without a >30° leftward axis shift. We found (1) that the prevalence of a left axis deviation during provocation testing was 18% and (2) that this left axis deviation was not explained by terminal conduction slowing in the right ventricular outflow tract (4th QRS-loop quartile: +17 ± 14 ms versus +13 ± 15 ms, nonsignificant) but was associated with a more proximal conduction slowing (1st QRS-loop quartile: +12[8;18] ms versus +8[4;12] ms, p < 0.001 and 3rd QRS-loop quartile: +12 ± 10 ms versus +5 ± 7 ms, p < 0.001). There was no important heterogeneity of the action potential morphology (no difference in the ventricular gradient), but a left axis deviation did result in a discordant repolarization (spatial QRS-T angle: 122[59;147]° versus 44[25;91]°, p < 0.001). Thus, although the development of the type-1 BrS-ECG is characterized by a terminal conduction delay in the right ventricle, BrS-patients with a left axis deviation upon sodium channel blocker provocation have an additional proximal conduction slowing, which is associated with a subsequent discordant repolarization. Whether this has implications for risk stratification is still undetermined.

Dormant conduction in the right ventricular outflow tract unmasked by adenosine in a patient with Brugada syndrome

2021 ◽  
Vol 32 (4) ◽  
pp. 1182-1186
Author(s):  
Tsukasa Kamakura ◽  
Josselin Duchateau ◽  
Frédéric Sacher ◽  
Pierre Jais ◽  
Michel Haïssaguerre ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Brugada Syndrome ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Dormant Conduction ◽  
The Right

scholarly journals Early Response after Catheter Ablation of the Epicardial Substrate in a Patient with Brugada Syndrome Can Be Predicted by High Precordial Leads

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Yae Min Park ◽  
Mi Sook Cha ◽  
Hanul Choi ◽  
Woong Chol Kang ◽  
Seung Hwan Han ◽  
...  
Keyword(s):  
Catheter Ablation ◽  
Brugada Syndrome ◽  
Low Voltage ◽  
Early Stage ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Early Response ◽  
Intercostal Space ◽  
The Right ◽  
Fourth Intercostal Space

A 52-year-old male with Brugada syndrome presented with repeated and appropriate shock from an implantable cardioverter defibrillator (ICD). Catheter ablation for substrate elimination targeting low-voltage, complex, and fractionated electrocardiograms and late potentials in the epicardial right ventricular outflow tract was successfully performed. Brugada phenotype in the right precordial leads from the third intercostal space disappeared in the early stage after catheter ablation and that from the standard fourth intercostal space disappeared later. He remained free from ventricular fibrillation over the next fourteen months. We suggest that this novel ablation strategy is effective in Brugada syndrome patients with ICD, and early response after catheter ablation can be predicted by high precordial leads.

scholarly journals Recent advances in cardiac catheterization for congenital heart disease

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 370 ◽  
Author(s):  
Sok-Leng Kang ◽  
Lee Benson
Keyword(s):  
Right Ventricular ◽  
Cardiac Catheterization ◽  
Congenital Heart ◽  
Imaging Techniques ◽  
Management Strategies ◽  
Three Dimensional ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Duct Occlusion ◽  
The Right

The field of pediatric and adult congenital cardiac catheterization has evolved rapidly in recent years. This review will focus on some of the newer endovascular technological and management strategies now being applied in the pediatric interventional laboratory. Emerging imaging techniques such as three-dimensional (3D) rotational angiography, multi-modal image fusion, 3D printing, and holographic imaging have the potential to enhance our understanding of complex congenital heart lesions for diagnostic or interventional purposes. While fluoroscopy and standard angiography remain procedural cornerstones, improved equipment design has allowed for effective radiation exposure reduction strategies. Innovations in device design and implantation techniques have enabled the application of percutaneous therapies in a wider range of patients, especially those with prohibitive surgical risk. For example, there is growing experience in transcatheter duct occlusion in symptomatic low-weight or premature infants and stent implantation into the right ventricular outflow tract or arterial duct in cyanotic neonates with duct-dependent pulmonary circulations. The application of percutaneous pulmonary valve implantation has been extended to a broader patient population with dysfunctional ‘native’ right ventricular outflow tracts and has spurred the development of novel techniques and devices to solve associated anatomic challenges. Finally, hybrid strategies, combining cardiosurgical and interventional approaches, have enhanced our capabilities to provide care for those with the most complex of lesions while optimizing efficacy and safety.

scholarly journals Longer Repolarization in the Epicardium at the Right Ventricular Outflow Tract Causes Type 1 Electrocardiogram in Patients With Brugada Syndrome

2008 ◽  
Vol 51 (12) ◽  
pp. 1154-1161 ◽  
Author(s):  
Satoshi Nagase ◽  
Kengo Fukushima Kusano ◽  
Hiroshi Morita ◽  
Nobuhiro Nishii ◽  
Kimikazu Banba ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Brugada Syndrome ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
The Right

scholarly journals Epicardial electrogram of the right ventricular outflow tract in patients with the brugada syndrome

2002 ◽  
Vol 39 (12) ◽  
pp. 1992-1995 ◽  
Author(s):  
Satoshi Nagase ◽  
Kengo Fukushima Kusano ◽  
Hiroshi Morita ◽  
Yoshihisa Fujimoto ◽  
Mikio Kakishita ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Brugada Syndrome ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
The Right
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