Revista de Medicina Desportiva (English) November 2018 - Page 18

Rev. Medicina Desportiva informa, 2018; 9(6):16-17. 25 years of the Brugada Syndrome By Basil Ribeiro, MD Dr Josep Brugada (JB) has given an interview to Medscape, con- ducted by Dr. Jorge González, about the 25 years of Brugada Syndrome (BS). It was published on 26/04/2018 and it includes a small historic review. He said that once he found a different electrocardiogram (EKG) in four persons that just had sudden dead. Then, with already eight identified cases, they made the first publication at JACC on 1992, which became a world alert to the medical community. Since then, the doctors got interest on patients with struc- turally normal hearts, but with an abnormal atypical EKG. JB refers that on that moment it was started a path for the study of the “genetically determined ionic channels disease”. They wanted to understand the cause of dead on those situations they call “idiopathic ventricular fibrillation”. There is an underneath genetic mechanism, “but not in all”, and this condition affects the “heart sodium channel, creating an ionic imbalance on certain areas of the heart, especially in the right ventric- ular epicardium”. The incidence is the same on males and on females, but “the women have more symp- toms”. The great concern of Dr. JB is the asymptomatic patient, because those that already were victims of this syndrome (“sudden dead or car- diogenic syncope”) are identified and should be protected. The strategy for the symptomatic patient is, on first place, “to remove the phenocopies of Brugada”, this, is, the EKGs that look like, but they aren’t BS. Then, he says, the confirmation should be made, either with “provocation test with a drug, like ajmaline, flecainide or procainamide”. The confirma- tion leads then to risk stratification. For Dr. JB, “if the patient is inducible it is recommended the implanta- tion of a defibrillator“. However, on patients with frequent ventricular arrhythmias, it is needed to have 16 november 2018 www.revdesportiva.pt their control with medication, like quinidine for example, which can’t be taken alone, because “just an epi- sode of ventricular fibrillation would kill the patient”. To determine the risk, Dr. JB indi- cates the following criteria: 1. Electrocardiogram 2. Provocative test 3. Induction test of the arrhythmia. The ablation with radiofrequency of the right ventricular epicardium is a good option and a recently devel- oped treatment for the more symp- tomatic patients, since the remove of the thin and localized layer of epicardium located at the exit of the right ventricle can restore the ionic balance between endocardium and epicardium at that level, with normalization of the electrocar- diogram, with negative provocative tests and the polymorphic ventricu- lar tachycardia won’t be induced in the electrophysiologic tests. And he concludes: “Genetically, the disease still exists, but we normalized all the phenotype expression”. Finally, Dr. JB in this interview believes that the future is on AND repair, on the efficient repair of the genes. However, until we can get there, “ablation will be the treatment for Brugada Syndrome”. Brugada’s family Dr. Josep Bru- gada was born on June, the 18 th , 1958 at Banyoles (Girona, Spain) and graduated on Medicine and Surgery at the University of Barce- lona in 1991. He is a pediatric cardi- ologist in charge of the arrhythmias department at the Sant Joan de Déu Hospital, in Barcelona. He is mas- ter on Integrated Health Services Management and he is specialist on Sports Medicine and Biology. He is an associated professor of medicine at the University of Barcelona since 2010 and he is the director of the project Difusión de los defibriladores semiautomáticos en Catalunya of the Millenni Foundation (Mútua Geral of Catalunya). He has been receiving several high prestige inter- national awards. In 1990 he was a finalist of a prize of Investigation of the American Society of Electrophys- iology and Stimulation (NASPE), in San Diego. Then, in 1991 he received a prize on cardiology of the Ameri- can College of Cardiology, in Atlanta. In 1999 he received the prize Fritz Ackher of the German Society of Cardiology and the prize Josep Tru- eta of the Medical Sciences Academy from Catalunya and Baleares. He has published more than 350 original papers and ten books, he had more than 450 presentations at several congresses and he organized about 50 courses. He has been cited in more than 9500 scientific papers. He was responsible for several doctoral thesis and he was the men- tor of several international fellow- ships about electrophysiology and arrhythmias. He was President of the European Heart Rhythm Association of Electrophysiology and he is respon- sible of the group Arrhythmias in the European Society of Cardiology’s Science Committee. Professor Pedro Brugada was born on August, the 11 th 1952 and he is responsi- ble for the Heart Rhythm Manage- ment Centre at the University of Ziejken- huis, in Brussels, where he is profes- sor of cardiology and physiology. In 2012 he received the gold medal of the European Society of Cardiology. He was in Portugal, on June the 6 th 2018, at Monte Real, during the Chal- lenges in Cardiology, as an invited by the President of the Portuguese Society of Cardiology, Dr. João Primo. He gave a memorial lecture with the title Brugada Syndrome 30 years of progress, celebrating 30 years of the first publication where Brugada Syn- drome is mentioned. This conference was presided by Dr. João Primo, one