HISTORY OF MEDICINE
Year : 2020 | Volume
: 21 | Issue : 2 | Page : 123--124
Evolution of brugada syndrome
Department of Cardiology, Heart Hospital, Hamad Medical Corporation, Doha, Qatar
Dr. Rachel Hajar
Sr. Consultant Cardiologist, Director of HH Publications and Executive Coordinator for Research; Director of Non-Invasive Cardiology (1981–2014), Heart Hospital, Hamad Medical Corporation, Doha
|How to cite this article:|
Hajar R. Evolution of brugada syndrome.Heart Views 2020;21:123-124
|How to cite this URL:|
Hajar R. Evolution of brugada syndrome. Heart Views [serial online] 2020 [cited 2022 Sep 27 ];21:123-124
Available from: https://www.heartviews.org/text.asp?2020/21/2/123/288325
Milestones in Our Understanding of Brugada Syndrome
1953 – Osher and Woff noticed the right bundle branch block (RBBB) with associated ST segment elevation in the right precordial lead on electrocardiogram (ECG) and considered as normal variant and having no relation to sudden cardiac death (SCD).
1975 – Calo reported an ECG triad: R' wave, SR segment elevation, and negative T wave in the right percordial leads. It was still considered a normal variant.
1980s – Center for Disease Control in Atlanta, USA, observed an abnormally high incidence of SCD in Asian refugees who immigrated to the USA from Thailand and were known in the country as “Lai Tai (death during sleep).”
1986 – Prof. Pedro Brugada receives his first patient with typical ECG features of Brugada – a Polish Caucasian child, who suffered several episodes of syncope with a sister who died of SCD.
1989 – In 1989, a patient with the characteristic ECG was described as being a carrier of early repolarization syndrome.
1991 – Brugada and Brugada presented an abstract in the NASPE meeting of a new clinical-cardiologic syndrome, characterized by the association of RBBB, persistent ST segment elevation, normal QT interval, and SCD.
1992 – The Brugada brothers presented the first description of the entity in the J Am Coll Cardiol.
1993–1995 – Several investigators describe the syndrome identified by the Brugada brothers with some critiquing the entity. The Brugada brothers responded in an Italian publication that the new entity they described has no underlying structural heart disease.
1996 – Two authors said in an article on studying the cellular basis of J wave in ECG that they used the eponym Brugada for the first time to describe the syndrome discovered 4 years earlier. Ikeda et al. also called the typical ECG signs of the new entity as “Brugada type.” Subsequently, several authors used the term “Brugada” when referring to the syndrome.
1998 – Identification of the genetic mutations in Brugada disease was identified and finally accepted as an entity. Chen identified three mutations in the SNC5A of chromosome 3p21–p24 which are responsible for Brugada disease.
1999 – Blazer included Brugada disease within the chapter of ion channel diseases or channelopathies. He showed that the affected channels in Brugada disease are primarily the fast sodium (Na) channel and secondarily the initial potassium outflow channel. Bessina et al. identified a single mutation in the Na channel, which was responsible for both Brugada disease and congenital long QT syndrome, that is, they share the same locus.
2000 – Noninvasive markers of value in the risk stratification of Brugada disease - Only high-resolution ECG has value in identifying a patient at high risk. High-resolution ECG has a sensitivity of 89%, a specificity of 50%, a positive predictive value of 70%, and a negative predictive value of 77% for the presence of late potentials. In a prospective study of 52 families, Priori et al. made the following conclusions:
Asymptomatic individuals with Brugada-type ECG pattern present a very low risk of SCDSymptomatic individuals with aborted SCD present 23% of the rate in a mean 33-month follow-upThe genetic mutation can be identified in 15% of the casesThe positive electrophysiologic study has a 50% of accuracyPharmacological tests have only a 35% of accuracy in asymptomatic carriers.
2001 – Major and minor criteria for diagnosing this entity were made. The authors proposed that the presence of a major criterion and a minor criterion one constitutes a diagnosis. Major criteria: ECG pattern in a patient without structural heart disease and a positive pharmacological test. Minor criteria: The presence of positive family history, syncope of unknown origin, documented episode of ventricular tachycardia/ventricular fibrillation (VF), and inducibility in the electrophysiologic and positive genetic study.
2002 – Genetic studies showed that unexplained nocturnal SCD syndrome, known as sudden unexplained nocturnal death syndrome, and Brugada disease are phenotypically, genetically, and functionally identical and allelic since both affect the same gene: SCN5A.
2003 – Atarashi et al. suggested that new ECG criteria would indicate high risk of VF in Brugada. Thus, the authors established the following:
S wave of duration ≥80 ms in V1 has a predictive value of 40.5% and a negative predictive value of 100% for VF with a 100% sensitivityST segment elevation in V2 of ≥80 ms measured from the J point has a positive predictive value of 37.8% and a negative predictive value of 100% for VF with a 100% sensitivity.
Both criteria are highly specific indicators for VF in this entity.
In a few years, the eponym, Brugada Syndrome, was used nearly unanimously by most investigators. There has been an exponential rise in the number of reported cases and a proliferation of papers serving to define the clinical, genetic, cellular, and molecular aspects of this disease.
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Conflicts of interest
There are no conflicts of interest.
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