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

[ARVD Symposium]

English - Spanish

Dear Andres,

Dr Wlodarska expressed a correct opinion, based on evidence based data.
You well know that the data which You enphasize on the "so called" Brugada
Syndrome are mostly derived from opinions and not epidemiological data. In
the far east detailed necropsy study and preventive cardiological examination
are not much available, so data on the underlying basis of sudden death are
presumed and not proved.
It must be clarified that an ECG is not a syndrome.
The discussed ECG is not so rare, but the syndrome, firtly described in
Italy, is rare as pointed out by more than an epidemiological study,
http://digilander.libero.it/martini_syndrome/page26.html
Recent surveys in Finland and Japan, are also more restrictive.
A big mistake to avoid is to presume (without any evidence based data), that
the unrare healthy people with this ECG will suffer of the syndrome, in
which case the "so called"  Brugada syndrome should be a problem bigger than 
AIDS, as pointed out by Dr Littmann in his letter: "The Brugada Numbers"
Circulation. 2003 May 1;107(18):e122;
Your statement "Brugada syndrome is believed to be responsible of 4-12% of
all SCD and around 20% of death with structural normal hearts".  has not
been proven by any well evidence based serie.
Moreover, almost all the patients died suddenly with this syndrome, and
submitted to a detailed necropsy study,  had a structural heart disease, of
the conduction system (as demonstrated decades ago by Kirschner), and of the
right ventricular Outflow tract,  as re-established also in a recent
abstract, unfortunately not yet published:  ?A multicenter histological
study of autopsied and biopsied specimens in BS?. Morimoto S. Eur Heart J
2003;24:147.
As a conclusion I would like to answer to   Jose Luiz Pantaleon Albornoz
Padilla from Malaga.
My point of view on the "so called" Brugada syndrome is that this syndrome
(not the ECG), is an organic heart disease closely linked to ARVD/C
My opinions can be read in the following article.

http://www.italheartj.org/pdf_files/Martini.pdf

Bortolo Martini  MD

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

Estimado Andres:
La Dra. Wlodarska expreso una opinion correcta, basandose en informacion
basada en evidencia. Usted bien sabe que la informacion que Ud. enfatiza en
el "llamado" Sindrome de Brugada deriva principalmente de opiniones y no de
datos epidemiologicos. En el lejano oriente, el estudio de la necropsia
detallada y el examen cardiologico preventivo no estan demasiado
disponibles, de modo que la informacion sobre las bases subyacentes de la
muerte subita es supuesta y no comprobada.
Debe quedar claro que un ECG no es un sindrome.
El ECG que se discute no es tan raro, pero el sindrome, primeramente
descrito en Italia, es raro como lo seniala mas de un estudio
epidemiologico,
http://digilander.libero.it/martini_syndrome/page26.html
Encuestas recientes en Finlandia y Japon tambien son mas restrictas.
Un gran error que hay que evitar es suponer (sin ninguna informacion basada
en evidencia) que las personas comunmente saludables con este ECG padecen el
sindrome, en cuyo caso el "llamado" Sindrome de Brugada deberia ser un
problema mas grande que el SIDA, como lo senialo el Dr. Littmann en su
carta: "Los Numeros de Brugada" Circulation.
2003 May 1;107(18):e122;
Su afirmacion de que se cree que el Sindrome de Brugada es responsable de un
4-12% de todas las MS y de alrededor del 20% de las muertes con corazones
estructuralmente normales, no se ha comprobado por ninguna serie basada en
evidencia.
Ademas, casi todos los pacientes que murieron subitamente con este sindrome,
y que fueron sometidos a un estudio detallado de necropsia, tenian una
cardiopatia estructural del sistema de conduccion (como lo demostro
Kirschner hace decadas) y del tracto de salida del ventriculo derecho, como
se reestablecio tambien en un resumen reciente, desafortunadamente no
publicado todavia: "A multicenter histological study of autopsied and biopsied 
specimens in BS". Morimoto S. Eur Heart J 2003;24:147.
Como conclusion, me gustaria responder a Jose Luiz Pantaleon Albornoz
Padilla de Malaga.
Mi punto de vista acerca del "llamado" Sindrome de Brugada es que este
sindrome (no el ECG), es una cardiopatia organica estrechamente vinculada
con la M/DAVD. Mis opiniones se pueden leer en el siguiente articulo.

http://www.italheartj.org/pdf_files/Martini.pdf

Bortolo Martini  MD

> 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