[ARVD-FORUM] Pathological difficulties to distinguish lipomatosis from true ARVD. Dr Martini

Sun Apr 10 14:41:29 ART 2005

English - Spanish

I think it may be that the audience could be interested by this old paper.
Many of the topics that we are discussing are long lasting!
Bortolo Martini

SEE THE FILES
http://www.arvd-symposium.org/files/Marini1.jpg
http://www.arvd-symposium.org/files/Martini2.jpg

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Pienso que la audiencia podría estar interesada por este viejo trabajo  
¡Muchos de los asuntos que estamos discutiendo vienen de larga data!
Bortolo Martini

VER LOS ARCHIVOS
http://www.arvd-symposium.org/files/Marini1.jpg
http://www.arvd-symposium.org/files/Martini2.jpg
 _____________________________________

>
> English - Spanish
>
> Dear Dr  Lorin de la Grandmaison,
> I suggest you to read carefully the paper we recently published:
> Basso C, Thiene G.  Adipositas cordis, fatty infiltration of the right
> ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a
> matter of fat? Cardiovasc Pathol. 2005 Jan-Feb;14(1):37-41.
>
> I think you will find some answers to your question. Take in mind that only
> a pathologist looking at hundred of hearts and sections per year of
> different diseases can reach the experience to differentiate between ARVC
> and fatty infiltration or dissociation which is not at all a
> cardiomyopathy. Unfortunately there are people which are not pathologists
> that introduced so many different terminologies as far as the morphology of
> the right ventricle that thay eventually increased the confusion about the
> real type ARVC.
>
> Best regards
>
> Yours truly
>
> Cristina Basso
>
> Cristina Basso, MD, PhD
> Professor, Cardiovascular Pathology
> Institute of Pathological Anatomy
> University of Padua Medical School
> via A. Gabelli, 61
> 35100 Padova- ITALY
>
> ---------------------
>
> Estimado Dr. Lorin de la Grandmaison:
>
> Le sugiero que lea cuidadosamente el trabajo que acabamos de publicar.
> Basso C, Thiene G.  Adipositas cordis, fatty infiltration of the right
> ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a
> matter of fat? Cardiovasc Pathol. 2005 Jan-Feb; 14(1): 37-41.
>
> Creo que encontrara algunas respuestas a sus preguntas. Tenga en cuenta que
> solo un patologo que ha observado cientos de corazones y cortes por anio de
> diferentes enfermedades, puede adquirir la experiencia para diferenciar
> entre DAVD e infiltracion o disociacion adiposa que no es para nada una
> miocardiopatia.
> Desdichadamente, existen muchas personas que no son patologos que
> introducen tanta terminologia diferente con respecto a la morfologia del
> ventriculo derecho, que eventualmente aumentan la confusion acerca del
> verdadero tipo de DAVD.
>
> Saludos cordiales,
>
> Cristina Basso
>
> Cristina Basso, MD, PhD
> Professor, Cardiovascular Pathology
> Institute of Pathological Anatomy
> University of Padua Medical School
> via A. Gabelli, 61
> 35100 Padova- ITALY
>
> _______________________________________
>
> > English - Spanish
> >
> > This is a problem that I like to discuss. I hope that Dr Dubner will be
> > able to show my slide and discussion based on systematic study of the
> > socalled "normal right ventricle"****. In my opinion, in addition to what
> > you have said Minor form of myocarditis can produce fibrosis and
> > subsequent changes similar to ARVD. This is why we are looking for a more
> > precise histologic marker.
> > We have seen and published (Kaplan HRS 2004 new journal soon in
> > pubmed...) that in Naxos disease there is a definite decrease in RV gap
> > junctions, however this feature has not been established in ARVD. However
> > this seems to me highly probable because C Guiraudon the pathologist has
> > demonstrated the presence desmosomes alteration in ARVD (Europ Heat J).
> > This was for me the stating point for the investigation in Naxos which is
> > recessive syndromic with a strong phenotypic presentation.
> > I also think that in you position in Garches you have the material of
> > deaths related to car accident providing an interesting pathologic
> > material of  so called "normal right ventricle".
> > I join the Fontaliran study on 85 specimen however I am sure that it will
> > be transferred properly on the Net.
> >
> > Guy
> >
> > SEE THE FILE
> > http://www.arvd-symposium.org/files/GradeFat.jpg
> >
> > SEE THE LECTURE "A mini-Atlas of ARVD Pathology"
> > http://www.arvd-symposium.org/lectures.shtml
> >
> > --------------------
> >
> > Este es un problema que me gusta discutir. Espero que el Dr. Dubner sea
> > capaz de mostrar mis diapositivas y discusion en base al estudio
> > sistematico del llamado "ventriculo derecho normal"****. En mi opinion,
> > ademas de lo que Ud. ha dicho, la forma menor de miocarditis puede
> > producir fibrosis y cambios subsiguientes similares a la DAVD. Esta es la
> > razon por la que estamos buscando un marcador histologico mas preciso.
> > Hemos visto y publicado (Kaplan HRS 2004 new journal soon in pubmed...)
> > que en la enfermedad de Naxos hay una disminucion definitiva en las
> > uniones "gap" del VD, sin embargo, esta caracteristica no se ha
> > establecido en la DAVD. Sin embargo, a mi me parece que esto es altamente
> > probable porque C. Guiraudon, el patologo, ha demostrado la presencia de
> > alteracion desmosomica en la DAVD (Europ Heat J). Para mi, este fue el
> > punto de inicio para la investigacion de Naxos, que es un sindrome
> > recesivo, con una presentacion fuertemente fenotipica.
> > Tambien creo que en su lugar en Garches, Ud. tiene el material de muertes
> > relacionadas con accidentes de autos, que ofrecen un interesante material
> > patologico del llamado "ventriculo derecho normal".
> > Me uni al estudio Fontaliran con 85 muestras, sin embargo, estoy seguro
> > de que se transferira adecuadamente a Internet.
> >
> > Guy
> >
> > VER EL ARCHIVO
> > http://www.arvd-symposium.org/files/GradeFat.jpg
> >
> > VER LA CONFERENCIA "A mini-Atlas of ARVD Pathology"
> > http://www.arvd-symposium.org/lectures.shtml
> >
> >  _______________________________________
> >
> > > English - Spanish
> > >
> > > Dear colleagues,
> > > Significant fat infiltration of the right ventricle which can be found
> > > in autopsy cases always raises the question of a possible underlying
> > > ARVD. The difficulty is that such right ventricular lipomatosis can be
> > > associated with small foci of fibrosis, especially if there is a small
> > > degree atheromatous stenosis, an obesity or a chronic alcohol disease.
> > > In such cases, what are the pathological criteria useful to make the
> > > differential diagnosis? And do you know if such lipomatosis can induce
> > > lethal arrhythmias even if is not a true ARVD?
> > >
> > > I thank you in advance for your answer.
> > >
> > > Best regards
> > > Geoffroy Lorin de la Grandmaison, MD
> > > Department of Pathology and Forensic Medicine, Raymond Poincaré
> > > Hospital, Garches, France
> > >
> > > ---------------------
> > >
> > > Estimados colegas:
> > > La infiltracion adiposa significativa del ventriculo derecho que puede
> > > encontrarse en los casos de autopsia siempre hace surgir la cuestion de
> > > una posible DAVD subyacente. La dificultad es que tal lipomatosis del
> > > ventriculo derecho puede asociarse con pequenios focos de fibrosis,
> > > especialmente si hay un grado menor de estenosis ateromatosa, obesidad
> > > o alcoholismo cronico. En tales casos, cuales son los criterios
> > > patologicos utiles para hacer un diagnostico diferencial? Y saben si
> > > tal lipomatosis puede inducir arritmias letales incluso si no es
> > > verdadera DAVD?
> > >
> > > Desde ya les agradezco su respuesta.
> > >
> > > Saludos cordiales,
> > >
> > > Geoffroy Lorin de la Grandmaison, MD
> > > Department of Pathology and Forensic Medicine, Raymond Poincaré
> > > Hospital, Garches, France