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