[ARVD-FORUM] ARVD and Brugada Syndrome. Dr. Wlodarska

[ARVD Symposium]

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


-- 
Dr. Sergio Dubner
Director

Dr. Edgardo Schapachnik
Director