Brugada syndrome, early repolarization syndrome/j-wave syndromes, and a subtype of idiopathic ventricular fibrillation: microstructural cardiomyopathies
DOI:
https://doi.org/10.36311/jhgd.v34.16300Keywords:
Brugada Syndrome, sudden cardiac deathAbstract
Brugada Syndrome is an inherited cardiac channelopathy with a high incidence of ventricular fibrillation and sudden cardiac death in patients with structurally normal hearts. Diagnosis is based on a characteristic electrocardiographic pattern (coved type ST-segment elevation ≥2 mm followed by a negative T-wave in ≥1 in the right precordial leads V1-V2) combined with an absence of gross structural abnormalities and several other criteria. The cornerstone of BrS diagnosis and definition, is its characteristic ECG pattern that can be present spontaneously or unmasked by drugs. This entity was described by the Brugada brothers in 1992 and belongs to a group of diseases known as inherited primary arrhythmia syndromes. The prevalence varies among regions and ethnicities, affecting mostly males. Despite several genes identified, SCN5A seems to be the most affected gene related BrS (≈ 30% of patients). The current main therapy is an implantable cardioverter-defibrillator, but radiofrequency catheter ablation has been recently reported as an effective new treatment.
Downloads
References
Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, Gussak I, LeMarec H, Nademanee K, Perez Riera AR, Shimizu W. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005 Feb 8;111(5):659-70.
Oreto G, Corrado D, Delise P, Fedele F, Gaita F, Gentile F, Giustetto C, Michelucci A, Padeletti L, Priori S. Doubts of the cardiologist regarding an electrocardiogram presenting QRS V1-V2 complexes with positive terminal wave and ST segment elevation. Consensus Conference promoted by the Italian Cardiology Society. Giornale italiano di cardiologia. 2010;11(11 Suppl 2):3s-22s.
Towbin JA, McKenna WJ, Abrams DJ, Ackerman MJ, Calkins H, Darrieux FC, Daubert JP, de Chillou C, DePasquale EC, Desai MY, Estes III NM. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart rhythm. 2019 Nov 1;16(11):e301-72.
Corrado D, Anastasakis A, Basso C, Bauce B, Blomström-Lundqvist C, Bucciarelli-Ducci C, Cipriani A, De Asmundis C, Gandjbakhch E, Jiménez-Jáimez J, Kharlap M. Proposed diagnostic criteria for arrhythmogenic cardiomyopathy: European Task Force consensus report. International Journal of Cardiology. 2024 Jan 15;395:131447.
Adytia GJ, Sutanto H. Brugada Phenocopy vs. Brugada Syndrome: Delineating the Differences for Optimal Diagnosis and Management. Current Problems in Cardiology. 2024 Apr 8:102566.
Zhang N, Liu T, Tse G, Yu S, Fu H, Xu G, Zhou C, Zhang C, Li G. Brugada phenocopy in a patient with acute pulmonary embolism presenting with recurrent syncope. Oxford Medical Case Reports. 2017 May;2017(5):omx014.
Bergamo D, Nelson C. Brugada pattern in adolescent with acute myocarditis due to SARS-CoV-2. Journal of the American College of Emergency Physicians Open. 2022 Oct;3(5):e12810.
Tsuchihashi K, Ueshima K, Uchida T, Oh-mura N, Kimura K, Owa M, Yoshiyama M, Miyazaki S, Haze K, Ogawa H, Honda T. Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction. Journal of the American College of Cardiology. 2001 Jul;38(1):11-8.
Chung EH. Brugada ECG patterns in athletes. Journal of Electrocardiology. 2015 Jul 1;48(4):539-43.
Gul EE, Haseeb S, Al Amoudi O, Baranchuk A. Brugada phenocopy associated with left ventricular aneurysm. Journal of Electrocardiology. 2018 Nov 1;51(6):963-5.
O’gara PT, Kushner FG, Ascheim DD, Casey Jr DE, Chung MK, De Lemos JA, Ettinger SM, Fang JC, Fesmire FM, Franklin BA, Granger CB. 2013 ACCF/AHA guideline for the management of ST-elevation myocardial infarction: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2013 Jan 29;127(4):529-55.
Awad SF, Barbosa-Barros R, de Sousa Belem L, Cavalcante CP, Riera AR, Garcia-Niebla J, Anselm DD, Baranchuk A. Brugada phenocopy in a patient with pectus excavatum: systematic review of the ECG manifestations associated with pectus excavatum. Annals of Noninvasive Electrocardiology. 2013 Sep;18(5):415-20.
Aizawa Y, Takatsuki S, Sano M, Kimura T, Nishiyama N, Fukumoto K, Tanimoto Y, Tanimoto K, Murata M, Komatsu T, Mitamura H. Brugada syndrome behind complete right bundle-branch block. Circulation. 2013 Sep 3;128(10):1048-54.
Madias JE, Sinha A, Agarwal H, Ashtiani R. ST-segment elevation in leads V (1)-V (3) in patients with LBBB. Journal of electrocardiology. 2001;34(1):87.
Levine HD, WANZER SH, MERRILL JP. Dialyzable currents of injury in potassium intoxication resembling acute myocardial infarction or pericarditis. Circulation. 1956 Jan;13(1):29-36.
Fang CF, Geng XU, Chen YX, WANG MY. Acute myocardial infarction mimicking squamous cell lung cancer with bone metastases due to hypercalcemia: a case report. Chinese medical journal. 2010 Feb 1;123(3):369-71.
Peltenburg PJ, Hoedemaekers YM, Clur SA, Blom NA, Blank AC, Boesaard EP, Frerich S, van den Heuvel F, Wilde AA, Kammeraad JA. Screening, diagnosis and follow-up of Brugada syndrome in children: a Dutch expert consensus statement. Netherlands Heart Journal. 2023 Apr;31(4):133-7.
Brugada J, Campuzano O, Arbelo E, Sarquella-Brugada G, Brugada R. Present status of Brugada syndrome: JACC state-of-the-art review. Journal of the American College of Cardiology. 2018 Aug 28;72(9):1046-59.
Monasky MM, Micaglio E, Ciconte G, Pappone C. Brugada syndrome: oligogenic or mendelian disease?. International Journal of Molecular Sciences. 2020 Mar 1;21(5):1687.
Campuzano Larrea O, Sarquella Brugada G, Cesar S, Arbelo E, Brugada Terradellas J, Brugada R. Update on Genetic Basis of Brugada Syndrome: Monogenic, Polygenic or Oligogenic?. International Journal of Molecular Sciences, 2020, vol. 21, núm. 19, p. 7155. 2020 Sep 28.
Monasky MM, Ciconte G, Anastasia L, Pappone C. Commentary: next generation sequencing and linkage analysis for the molecular diagnosis of a novel overlapping syndrome characterized by hypertrophic cardiomyopathy and typical electrical instability of Brugada syndrome. Frontiers in Physiology. 2017 Dec 12;8:1056.
Monasky MM, Pappone C, Piccoli M, Ghiroldi A, Micaglio E, Anastasia L. Calcium in Brugada syndrome: questions for future research. Frontiers in Physiology. 2018 Aug 10;9:1088.
Mango R, Luchetti A, Sangiuolo R, Ferradini V, Briglia N, Giardina E, Ferrè F, Citterich MH, Romeo F, Novelli G, Sangiuolo F. Next generation sequencing and linkage analysis for the molecular diagnosis of a novel overlapping syndrome characterized by hypertrophic cardiomyopathy and typical electrical instability of Brugada syndrome. Circulation Journal. 2016 Mar 25;80(4):938-49.
Pappone C, Micaglio E, Locati ET, Monasky MM. The omics of channelopathies and cardiomyopathies: what we know and how they are useful. European Heart Journal Supplements. 2020 Nov;22(Supplement_L):L105-9.
Pappone C, Monasky MM, Micaglio E, Ciconte G. Right ventricular electromechanical abnormalities in Brugada syndrome: is this a cardiomyopathy?. European Heart Journal Supplements. 2020 Jun;22(Supplement_E):E101-4.
Aurlien D, Leren TP, Taubøll E, Gjerstad L. New SCN5A mutation in a SUDEP victim with idiopathic epilepsy. Seizure. 2009 Mar 1;18(2):158-60.
Chahal CA, Salloum MN, Alahdab F, Gottwald JA, Tester DJ, Anwer LA, So EL, Murad MH, St Louis EK, Ackerman MJ, Somers VK. Systematic review of the genetics of sudden unexpected death in epilepsy: potential overlap with sudden cardiac death and arrhythmia-related genes. Journal of the American Heart Association. 2020 Jan 7;9(1):e012264.
Shalash A, Abu-Rmeileh NME, Kelly D, Elmusharaf K. Descriptive Overview of Adolescent Health Indicators in Humanitarian Settings: A Cross-Country Analysis. J Hum Growth Dev. 2024; 34(2):198-209.DOI: http://doi.org/10.36311/jhgd.v34.16301
Pedraza DF, Silva NS, Salaroli LB. Sleep habits of preschool children after the covid-19 lockdown in a municipality in paraíba, brazil. J Hum Growth Dev. 2024; 34(2):210-220. DOI: http://doi.org/10.36311/jhgd.v34.15838
Machado PCR, Filho AC, Coutinho BPNC, Manhabusque KV, Smiderle FRN. Food supplementation policy for pregnant women: analysis of coverage in Brazilian regions in the light of Covid-19. J Hum Growth Dev. 2024; 34(2):221-231. DOI: http://doi.org/10.36311/jhgd.v34.14857
Neto LCBS, Vieira TS, Souza MLM, Corrêa CHP, Borloti EB, Ferro PL, Silva AMA , Lopes-Júnior LC. Abdominal obesity is associated with stress levels among public safety personnel. J Hum Growth Dev. 2024; 34(2):232-243. DOI: http://doi.org/10.36311/jhgd.v34.15430
Lima Silva JMF, Mello JB, Cirilo-Sousa MS, Caldas GFR, Trigueiro ESO, Figueiredo FWS. Analyzing the adolescents’ impaired physical health: prevalence and associated factors in more than 100,000 brazilian schoolchildren. J Hum Growth Dev. 2024; 34(2):244-254. DOI: http://doi.org/10.36311/jhgd.v34.16302
Bueno GN, Tavares H, Macedo LR, Neto ETS. Internet addiction in late adolescence: profile and patterns of use. J Hum Growth Dev. 2024; 34(2):255-267. DOI: http://doi.org/10.36311/jhgd.v34.15753
Dias E, Costa WP, Fernandes MSV, Valente SN, Noll PRES, Noll M. Teachers’ quality of life perception during the covid-19 pandemic: a systematic review protocol. J Hum Growth Dev. 2024; 34(2):268-277. DOI: http://doi.org/10.36311/jhgd.v34.15837
Soares KKS, Hisatugu WH, Souza FM, Maciel ELN, Prado TN. Analysis of the Vulnerability Profile of tuberculosis co-infection in people living with HIV. J Hum Growth Dev. 2024; 34(2):278-285. DOI: http://doi.org/10.36311/jhgd.v34.15778
Cola JP, Pinto SA, Souza JS, Hertel JF, Galavote HS, Prado TN, Maciel ELN. Factors associated with abandonment of tuberculosis treatment: a cross-sectional study between 2014 and 2019. J Hum Growth Dev. 2024; 34(2):286-295. DOI: http://doi.org/10.36311/jhgd.v34.14098
Orlandi IS, Pestana ABC, Aguiar BLP, Sena ABE, Messetti PAS, Macedo Jr H, Leitão FNC, Sousa OF, Abreu LC. Time series of mortality from stroke in the adult population residents of the state of amazonas from 2000 to 2021. J Hum Growth Dev. 2024; 34(2):296-304. DOI: http://doi.org/10.36311/jhgd.v34.16304
Pinheiro CG, Pinto MVM, Baron MV, Sampaio AR, Fortuny E, Gomes OP, Silva VF, Júnior JGAS, Correa JA. Analysis of the mac® healing acceleration methodology with the use of propolis and toluidine blue in the healing of lower limb ulcers. J Hum Growth Dev. 2024; 34(2):305-314. DOI: http://doi.org/10.36311/jhgd.v34.15499
Silva LB, Moura MS, Madeira FP, Florencio WG, Júnior SLP, Ribeiro MAL, Oliveira J, Meneguetti DUO. Knowledge of residents about chagas disease and its vectors in a municipality of juruá, amazonas J Hum Growth Dev. 2024; 34(2):315-327. DOI: http://doi.org/10.36311/jhgd.v34.16224
Cunha UMM, Lucena KDT, Sousa LVA. Covid-19 pandemic and its impact on breast cancer screening in Brazil. J Hum Growth Dev. 2024; 34(2):328-341. DOI: http://doi.org/10.36311/jhgd.v33.15810
Sette RBT, Morais TC, Rêgo Costa ACSR, Sousa LVA, Zangirolami-Raimundo J, Daboin BEG, Leite HF, Cavalcanti MPE, Raimundo RD. Aquatic high-intensity interval training improves cardiometabolic profile and physical fitness in active middle-age and older adults: quasi-randomized clinical trial study. J Hum Growth Dev. 2024; 34(2):342-353.DOI: http://doi.org/10.36311/jhgd.v34.16305
Reis JRG, Gonçalves DF, Tonello MGM, Magalhães FH, Massa M, Silva AP, Moraes IAP, Pimentel R, Martinez JP, Monteiro CBM. Analysis of heart rate variability and anthropometric measurements to compare obesity classes II and III in patients undergoing bariatric surgery. J Hum Growth Dev. 2024; 34(2):354-365. DOI: http://doi.org/10.36311/jhgd.v34.16331
Macedo Neves SAV, Moreno NP, Baquette RF, Maia JRS, Gonçalves DM, Matos IS, Neto AND. Ocular paracoccidioidomycosis in the western amazon: a case report. J Hum Growth Dev. 2024; 34(2):366-370. DOI: http://doi.org/10.36311/jhgd.v34.16335
Downloads
Published
Issue
Section
License
Copyright (c) 2024 Pérez-Riera AR, Nikus K, Baranchuk A, Brugada P
This work is licensed under a Creative Commons Attribution 4.0 International License.
