[ARVD-FORUM] Some more remarks. Dr. Zareba

[ARVD Symposium]

English - Spanish

Dear Andres,

I am fully aware of the data you quoted and I want to emphasize that ARVD is
most likely underdiagnozed entity. recognized mostly by tertiary centers.
Increasing awareness about ARVD, through variety of educational programs
including this symposium, will contribute to more frequent diagnosis of this
disorder. Looking back 10 years ago, diagnosis of LQTS was quite infrequent,
decade later physicans know it well and diagnose it more frequently. The
same pattern hopefully will be followed by ARVD.

The paper by Dr. Wlodarska is of major importance reconfirming familial form
of the disorder in the majority of cases with various penetrance among
studied families. This observation further prompts physicians to evaluate
and monitor family members of ARVD probands.

The data from autopsy of athletes are very intiguing, but could represent
biased sample. There is a theory that vigorous physical activity might
contibute to expression of otherwise silent ARVD genes. Overstrech of right
ventricle (both ventricles) during athletic activities might be the factor
turnning on clinical form of ARVD. More studies are needed to determine this
potential association.

Best regards

Wojciech Zareba

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

Estimado Andres,

Soy totalmente consciente de la informacion que cita y deseo enfatizar que
la DAVD es muy probablemente, una entidad subdiagnosticada, reconocida
principalmente por centros terciarios. Una mayor conciencia sobre la DAVD, a
traves de una variedad de programas educativos incluyendo este simposio,
contribuira a una diagnostico mas frecuente de este trastorno. Mirando hacia
atras, hace 10 anios el diagnostico de LQTS era bastante poco frecuente;
pero una decada mas tarde los medicos la conocen bien y la diagnostican con
mayor frecuencia. Esperemos que se siga el mismo patron con la DAVD.

El trabajo de la Dra. Wlodarska es sumamente importante, y reconfirma la
forma familiar de este trastorno en la mayoria de los casos con penetrancias
diversas en las familias estudiadas. Esta observacion anima aun mas a los
medicos a evaluar y monitorear a los parientes de los casos testigos con
DAVD.

Los datos de las autopsias de los atletas son muy intrigantes, pero pueden
representar una muestra parcializada. Hay una teoria que establece que la
actividad fisica vigorosa puede contribuir con la expresion de los genes
DAVD que de lo contrario permanecen silentes. El sobreestiramiento del
ventriculo derecho (ambos ventriculos) durante las actividades atleticas
puede ser el factor que activa la forma clinica de la DAVD. Se necesitan mas
para determinar esta asociacion potencial.

Saludos cordiales,

Wojciech Zareba

>
> 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

-- 
Dr. Sergio Dubner
Director

Dr. Edgardo Schapachnik
Director