{"title":"布鲁加达综合征:2017年更新。(RCD代码:V-1A.1)","authors":"Paweł Rubiś","doi":"10.20418/JRCD.VOL3NO4.302","DOIUrl":null,"url":null,"abstract":"Brugada Syndrome (BrS) is traditionally considered a primary channelopathy, most commonly due to reduced inward sodium current with an increased risk of syncope and sudden cardiac death (SCD). According to the recent guidelines, BrS is diag‐ nosed in patients with ST‐segment elevation with type 1 morphol‐ ogy among the right precordial leads V1 and V2, occurring either spontaneously or after provocative drug test with intravenous administration of Class I antiarrhythmic drugs. Moreover, BrS is diagnosed in patients with type 2 or type 3 ST‐segment elevation in ≥1 lead among the right precordial leads when a provocative drug test with intravenous administration of Class I antiarrhyth‐ mic drugs induces a type I ECG morphology. Risk stratification of SCD is the most important aspect of the concise management of those patients. Importantly, once considered pure arrhyth‐ mic syndrome, nowadays, there is growing understanding that some structural abnormalities may be present in BrS. Therefore, imaging is actively investigated in this field. Brugada syndrome can be considered as a rare disease, with the prevalence of 1 in 1000 to 1 in 10 000, being more frequent in south‐east Asia than in the western countries. Thus, this topic has been already explored in the Journal of Rare Cardiovascular Disease (see references be‐ low). The purpose of this Review is to update the Readers with the main developments in this entity.","PeriodicalId":37488,"journal":{"name":"Journal of Rare Cardiovascular Diseases","volume":"4 1","pages":"108-109"},"PeriodicalIF":0.0000,"publicationDate":"2017-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"1","resultStr":"{\"title\":\"Brugada syndrome: 2017 update. (RCD code: V-1A.1)\",\"authors\":\"Paweł Rubiś\",\"doi\":\"10.20418/JRCD.VOL3NO4.302\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Brugada Syndrome (BrS) is traditionally considered a primary channelopathy, most commonly due to reduced inward sodium current with an increased risk of syncope and sudden cardiac death (SCD). According to the recent guidelines, BrS is diag‐ nosed in patients with ST‐segment elevation with type 1 morphol‐ ogy among the right precordial leads V1 and V2, occurring either spontaneously or after provocative drug test with intravenous administration of Class I antiarrhythmic drugs. Moreover, BrS is diagnosed in patients with type 2 or type 3 ST‐segment elevation in ≥1 lead among the right precordial leads when a provocative drug test with intravenous administration of Class I antiarrhyth‐ mic drugs induces a type I ECG morphology. Risk stratification of SCD is the most important aspect of the concise management of those patients. Importantly, once considered pure arrhyth‐ mic syndrome, nowadays, there is growing understanding that some structural abnormalities may be present in BrS. Therefore, imaging is actively investigated in this field. Brugada syndrome can be considered as a rare disease, with the prevalence of 1 in 1000 to 1 in 10 000, being more frequent in south‐east Asia than in the western countries. Thus, this topic has been already explored in the Journal of Rare Cardiovascular Disease (see references be‐ low). The purpose of this Review is to update the Readers with the main developments in this entity.\",\"PeriodicalId\":37488,\"journal\":{\"name\":\"Journal of Rare Cardiovascular Diseases\",\"volume\":\"4 1\",\"pages\":\"108-109\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2017-10-16\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Rare Cardiovascular Diseases\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.20418/JRCD.VOL3NO4.302\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Rare Cardiovascular Diseases","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.20418/JRCD.VOL3NO4.302","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
Brugada Syndrome (BrS) is traditionally considered a primary channelopathy, most commonly due to reduced inward sodium current with an increased risk of syncope and sudden cardiac death (SCD). According to the recent guidelines, BrS is diag‐ nosed in patients with ST‐segment elevation with type 1 morphol‐ ogy among the right precordial leads V1 and V2, occurring either spontaneously or after provocative drug test with intravenous administration of Class I antiarrhythmic drugs. Moreover, BrS is diagnosed in patients with type 2 or type 3 ST‐segment elevation in ≥1 lead among the right precordial leads when a provocative drug test with intravenous administration of Class I antiarrhyth‐ mic drugs induces a type I ECG morphology. Risk stratification of SCD is the most important aspect of the concise management of those patients. Importantly, once considered pure arrhyth‐ mic syndrome, nowadays, there is growing understanding that some structural abnormalities may be present in BrS. Therefore, imaging is actively investigated in this field. Brugada syndrome can be considered as a rare disease, with the prevalence of 1 in 1000 to 1 in 10 000, being more frequent in south‐east Asia than in the western countries. Thus, this topic has been already explored in the Journal of Rare Cardiovascular Disease (see references be‐ low). The purpose of this Review is to update the Readers with the main developments in this entity.
期刊介绍:
Journal of Rare Cardiovascular Diseases (JRCD) is an international, quarterly issued, peer-reviewed, open access, online journal that keeps cardiologists and non-cardiologists up-to-date with rare disorders of the heart and vessels. The Journal publishes fine quality review articles, original, basic and clinical sciences research papers, either positive or negative, case reports and articles on public health issues in the field of rare cardiovascular diseases and orphan cardiovascular drugs. Topics of interest include, but are not limited to the following areas: (1) rare diseases of systemic circulation (2) rare diseases of pulmonary circulation (3) rare diseases of the heart (cardiomyopathies) (4) rare congenital cardiovascular diseases (5) rare arrhythmogenic disorders (6) cardiac tumors and cardiovascular diseases in malignancy (7) cardiovascular diseases in pregnancy (8) basic science (9) quality of life