[ARVD-FORUM] ARVD and Brugada Syndrome. Dr. Perez Riera

Mon Apr 4 19:54:00 ART 2005

English - Portuguese

Dear Dr Wlodarska, 
I disagree with your estimative. Why?  Because the ECG pattern in Brugada 
Syndrome  can be dynamic and is often concealed, it is difficult to estimate 
the true prevalence of the disease in the general population.

This entity causes 4 to 10 SCD per 10,000 inhabitants per year in areas like
Thailand and Laos (Southeast Asia). It is particularly frequent among young
men (under the age of 40) of Asian origin. In these countries, the disease
represents the leading cause of death in young adult men (Brugada J, Brugada
P, Brugada R. The syndrome of right bundle branch block ST segment
elevation in V1 to V3 and sudden death-the Brugada syndrome. Europace 1999;
1:156-66.). Brugada syndrome deaths are second only to automobile accidents
as a cause of death among young adults men in some countries around the
world.

The annual mortality rate in Thailand for this subgroup the. prevalence of
the disease was estimated to be 26 to 38 deaths per 100,000
inhabitants (Nademanee KK, Veerakul G, Nimmannit, S, et.al. Arrhytmogenic
marker for the sudden unexplained death syndrome in Thai men. Circulation.
1997; 96:2595-2600.). Brugada syndrome is believed to be responsible for
4-12% of all SCD and around 20% of deaths in patients with structurally
normal hearts (Juang JM, Huang SK.Brugada syndrome--an under-recognized
electrical disease in patients with sudden cardiac death.Cardiology. 2004;
101:157-169.).

Best regard

Andrés Ricardo Pérez Riera

-------------------------------------------------

Prezado  Dr Wlodarska   
Eu discordo com sua estimativa. Porque?  Porque o padrão ECG na síndrome de 
Brugada pode ser dinâmico e com freqüência oculto sendo difícil assim estimar 
a verdadeira prevalência da enfermidade na população geral.

Esta entidade causa 4 a 10 SCD por 10,000 habitantes por ano em áreas como
Tailândia e Laos (Sudeste asiático) .É particularmente freqüente entre
homens jovens abaixo dos 40 anos de origem asiático (Brugada J, Brugada P,
Brugada R. The syndrome of right bundle branch block ST segment  elevation
in V1 to V3 and sudden death-the Brugada syndrome. Europace 1999;
1:156-66.)..  Em estes paises representa a principal causa de morte natural
entre homens jovens depôs dos acidentes de tránsito. Acredita-se que a
síndrome seja responsável por 4% a 12% das MCS e ao redor de 20% das mortes
em pacientes com coração estruturalmente normal. (Juang JM, Huang SK.Brugada
syndrome--an under-recognized electrical disease in patients with sudden
cardiac death.Cardiology. 2004; 101:157-169.).

Saludacoes

Andres Ricardo Pérez Riera.

>
> English - Spanish
>
> Dear Prof. Zareba, Dr Perez Riera, Colleagues,
>
> Please find enclosed an abstract (see below) of our article ARRHYTHMOGENIC
> RIGHT VENTRICULAR CARDIOMYOPATHY, BRUGADA  SYNDROME AND MULTILEVEL CARDIAC
> CONDUCTION DISEASE - CASE REPORT published in Folia Cardiologica 2003; 10:
> 837-845 describing the rare situation where ARVD coexists with Brugada
> Syndrome. In my opinion this case represents one of the clinical forms of
> ARVD.
>
> Differential diagnosis of ARVD, ARVD with Brugada Syndrome and Brugada
> Syndrome is analyzed in the enclosed table.
> http://www.arvd-symposium.org/files/Wlodarska_tab.html
>
> In our experience ARVD is far more frequent  than Brugada Syndrome. Our
> group
> of ARVC patients consists of about 200 cases while Brugada Syndrome was
> observed only in 5 patients. Most of our ARVC patients were recruited from
> Warsaw and Silesia area where the biggest Polish arrhythmologic centers 
> are situated, so the prevelance of the disease is still unknown and is
> probably underestimated in other regions of Poland.
>
> Yours sincerely,
>
> ----------------------
>
> Estimados Prof. Zareba, Dr. Perez Riera y colegas:
>
> Por favor, les envio en el adjunto (ver abajo) un resumen de nuestro
> articulo ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY, BRUGADA  SYNDROME
> AND MULTILEVEL CARDIAC CONDUCTION DISEASE - CASE REPORT publicado por Folia
> Cardiologica 2003; 10: 837-845, que describe la rara situacion en la que la
> DAVD coexiste con el Sindrome de Brugada. En mi opinion este caso
> representa una de las formas clinicas de DAVD.
>
> El diagnostico diferencial de la DAVD, la DAVD con Sindrome de Brugada y el
> Sindrome de Brugada se analiza en la tabla adjunta.
> http://www.arvd-symposium.org/files/Wlodarska_tab.html
>
> En nuestra experiencia, la DAVD es mucho mas frecuente que el Sindrome de
> Brugada. Nuestro grupo de pacientes con DAVD consiste en aproximadamente
> 200 casos, mientras que el Sindrome de Brugada se observo solo en 5
> pacientes. La mayoria de nuestros pacientes con M/DAVD fueron reclutados en
> areas de Varsovia y Silesia, donde se encuentran los mayores centros de
> arritmologia de Polonia, por lo que la prevalencia de la enfermedad es aun
> desconocida y probablemente se subestime en otras regiones de Polonia.
>
> Atentamente,
>
> Katarzyna Wlodarska, MD, PhD.
>
> -----------------------------
>
> ABSTRACT
>
> ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY, BRUGADA  SYNDROME AND
> MULTILEVEL CARDIAC CONDUCTION DISEASE - CASE REPORT
> EK Wlodarska, A Wojcik, F Walczak, et al.
>
>
> 21 year old man with the history of palpitation and pre-syncope was
> admitted because of automaticity and conduction disturbances (sinus pauses,
> atrio-ventricular block of  I and II degree, RBBB) and recurrent atrial
> tachycardia.  Arrhythmogenic right ventricular cardiomyopathy was diagnosed
> on the basis of noninvasive tests (RBBB, positive late potentials,
> localized kinetic abnormalities of the right ventricle). EP study showed
> multilevel conduction disease. After Ajmaline infusion ST segment elevation
> in right ventricular leads was observed. In 55th second after the infusion
> monomorphic ventricular tachycardia and then ventricular flutter and
> fibrillation was observed,  suggesting Brugada syndrome. Additionally TILT
> was performed revealing mixed vaso-vagal syndrome. ICD with DDD function
> was implanted. Differential diagnosis between arrhythmogenic right
> ventricular cardiomyopathy and Brugada syndrome was discussed.
>
> Table 1.
> Differential diagnosis of ARVC and Brugada syndrome
> http://www.arvd-symposium.org/files/Wlodarska_tab.html
>
> Fig. 1. Echocardiography. Modified apical four - chamber  view. Bulges of
> apex of the right ventricle (arrow). Thickening of endocardium in the areas
> of moderator band and intraventrucular septum (arrows). PP - right atrium,
> PK - right ventricle, LP - left atrium, LK - left ventricle
> http://www.arvd-symposium.org/files/Wlodarska_fig1.jpg
>
> Fig. 2. Electrocardiogram. Sinus rhythm. First grade of A-V block. Right
> bundle branch block (A). After ajmalin infusion in leads V1-V4 ST segment
> elevation, followed by ventricular fluttering (B) and finally evolving into
> ventricular fibrillation (C)
> http://www.arvd-symposium.org/files/Wlodarska_fig2A.jpg
> http://www.arvd-symposium.org/files/Wlodarska_fig2B.jpg
> http://www.arvd-symposium.org/files/Wlodarska_fig2C.jpg