[ARVD-FORUM] Some more remarks. Dr. Perez Riera

[Dr. Edgardo Schapachnik]

English - Portuguese

Dear Dr .Zareba,
Here Andrés Ricardo Pérez Riera form São Paulo Brazil. 
Prevalence of ARVC/D is estimated as 1/5000 (Czarnowska E, Wlodarska EK, 
Zaleska T.  Arrhythmogenic right ventricular cardiomyopathy (dysplasia): 
etiology, clinical presentation, diagnosis and treatment Kardiol Pol. 2003; 
58:58-63.)

The prevalence is estimated at 0.4% depending on geographic circumstances. 
(Hagenah G, Andreas S, Konstantinides S. Accidental left ventricular 
placement of a defibrillator probe due to a patent foramen ovale in 
arrhythmogenic right ventricular dysplasia. Acta Cardiol. 2004; 59:449-451.) 
like Brugada Syndrome. The entity is endemic in Veneto region (Italy), Nova 
Scotia and Naxus Greek island (recesive form). The incidence of SCD is 
approximately 2.5% a year. In the athletes group < 35 years, ARVC/D is the 
more important cause of death in Europe. Studies have shown that ARVC/D is a 
significant cause of SCD among young athletes despite, circumstances of death 
are various, but 75.6% occurred during everyday life events (at home, 63.1%; 
in the street, 6.6%; or at work, 6.1%); only 3.5% occurred during sports 
activity. (Tabib A, Loire R, Chalabreysse L, et al Circumstances of death and 
gross and microscopic observations in a series of 200 cases of sudden death 
associated with arrhythmogenic right ventricular cardiomyopathy and/or 
dysplasia. Circulation. 2003; 108:3000-3005).

 ARVC/D may account for as many as 5% of unexpected SCD under the age of 65 
and 3-4% of SCD during sports. 

ARVC/D is an important cause of SCD in the young.  

Best regard

Andres

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

Portuguese

Prezado Dr Zareba: 
Aqui Andrés Ricardo Pérez Riera de São Paulo Brasil. A prevalencia da DAVD se 
ha estimando en 1/5000, o 0.4% dependendo de circuntâncias geográficas. Como 
a sindrome de Brugada  tiene areas endémicas, como na região de Veneto na 
Italia nova Escocia e na ilha grega de Naxos (na forma recessiva). Entre 
jovens atletas menores de 35 anos é a principal causa de morte na Europa. 
Estudos tem mostrado que a DAVD é uma causa significativa de MCS entre 
atletas porem as circunstacias da morte são varias 75,6% ocorrem durante o 
dia no lar; 63% na rua;  6.6% no trabalho 6,1% e apenas em  3,5% durante a 
atividade esportiva. A la DAVD se le pode atribuir tanto quanto 5% das mortes 
subitas inesperada abaixo de 65 anos e 3-4% das MCS durante esportes. A DAVD 
é uma importante causa de morte no jovem.

Saludacoes

Andres.

> English - Spanish
> Dear Guy,
>
> We definitely value your opinion and it could well be that the disease is
> largely underestimated. At least when we compare with number of cases seen
> clinically of LQTS, HCM, even Brugada syndrome, ARVD is far behind, very
> rare.
>
> But, the virtual symposium is exactly good forum to express such opinions
> and
> we appreciate you writing a paragraph on it and we will send your comment
> to all participants.
>
> Best regards
>
> Wojciech
>
> ----------------------
>
> Estimado Guy:
>
> Definitivamente valoramos tu opinion y bien podria ser que la enfermedad
> este enormemente menospreciada. Por lo menos cuando la comparamos con el
> numero de casos vistos clinicamente de LQTS, CMH, incluso del Sindrome de
> Brugada, la DAVD esta muy por detras con respecto a las otras, por eso es
> rara.
>
> Pero el Simposio Virtual es exactamente el foro para expresar tales
> opiniones, y apreciamos que escribas un parrafo al respecto. Nosotros
> enviaremos tus comentarios a todos los participantes.
>
> Saludos cordiales,
>
> Wojciech
>
>
> t _______________________________________
>
> > English - Spanish
> >
> > Dear Wojciech
> >
> > I do not agree with the statement that ARVD is a rare disease !
> >
> > The histologic substrate of RVD is found in 3.7 % of the general
> > population
> >
> > only few patients are referred to tertiary centers because of
> > arrhythmias.
> >
> > Around 3-5 cases each month are seen in Paris in the Forensic Medicine
> > Institution
> >
> > 10 cases are studied each day !
> >
> > In addition many patients have concealed RV dysfunction. This is visible
> > only in case of independent LV failure
> > Therefore ARVD and Fat Dissociation Syndrome (see my histopathologic
> > plates) are important pronostic factors as recently reported.
> >
> > Best regards
> >
> > Guy
> >
> > ----------------
> >
> > Estimado Wojciech
> >
> > No coincido con la idea de que la DAVD es una enfermedad rara!
> > El sustrato histologico de DVD se encuentra en el 3,7 % de la poblacion
> > general, solo unos pocos pacientes son derivados a centros terciarios
> > debido a arritmias.
> >
> > En Paris se ven alrededor de 3-5 casos por mes; en la Forensic Medicine
> > Institution se estudian 10 casos cada dia!
> >
> > Ademas, muchos pacientes tienen disfuncion oculta del VD. Esto solo es
> > evidente en el caso de insuficiencia independiente del VI.
> > Por lo tanto, la DAVD y el Sindrome de Disociacion de Adiposidad (ver mis
> > placas histopatologicas) son factores de pronostico importantes como se
> > informo recientemente.
> >
> > Saludos cordiales,
> >
> > Guy
>
> _______________________________________________
> Arvd-forum mailing list
> Arvd-forum en ishne.org
> http://www.ishne.org/mailman/listinfo/arvd-forum