Fwd: [ARVD-FORUM] Naxos' disease in dogs? Dr. Fontaine
ARVD Symposium
info at arvd-symposium.org
Fri Apr 8 14:09:56 ART 2005
English - Spanish
Apoptosis was published by Dr Mallat from my group in the New England J M
See in my biography
Guy Fontaine
----------------
Apoptosis fue publicado por el Dr. Mallat, de mi gruoi, en el New England J M
Vean en mi biografia
Guy Fontaine
_______________________________________
>
> English - Spanish
>
> Dear Dr Carlos H. Lightowler due to scant existing animal models, the
> etiology and pathogenesis of ARVC/D remain unknown or incompletely solved
> (the pathophysiological mechanisms have been impaired by lack of suitable
> animal models. To this date, three animals have been used for this purpose:
>
> 1) COMMON DOMESTIC CAT: Fox et al have identified a ctinically
> relevant cardiomyopathy. Such cardiomyopathy closely resembles human
> ARVC/D. The relevance of this unique feline model of human disease will be
> important in the definition of pathogenesis and in the research of
> mechanisms accountable for the progression of the disease in ARVC/D. (Fox
> PR, Maron BJ, Basso C, Liu SK, Thiene G. Spontaneously occurring
> arrhythmogenic right ventricular cardiomyopathy in the domestic cat: A new
> animal model similar to the human disease. Circulation. 2000;
> 102:1863-1870).
> Recently Harvey et al. sowed two cat affected. On 24-hour (Holter)
> electrocardiogram recording revealed complete atrioventricular block and
> multiform ventricular ectopics in both cats, with the addition of
> ventricular tachycardia, ventricular bigeminy and R-on-T phenomenon in one
> of them. (Harvey AM, Battersby IA, Faena M, Fews D, Darke PG, Ferasin
> L.Arrhythmogenic right ventricular cardiomyopathy in two cats. J Small Anim
> Pract. 2005; 46:151-156.). One cat had also experienced previous episodes
> of syncope. On echocardiography, the right ventricle and atrium were
> massively dilated and hypokinetic. The survival times of the cats were
> three days and 16 days following diagnosis. Histopathology in one case
> revealed fibro-fatty infiltration of the myocardium, predominantly
> affecting the right ventricular free wall.
>
> 2) BOXER DOGS: this seems to be the ideal animal model to study the
> human disease, since it reproduces spontaneously and transmits genetically
> the same etiopathogenic, histopathological, anatomopathological and
> clinical features as in humans. (Basso C, Fox PR, Meurs KM, Towbin JA,
> Spier AW, Calabrese F, Maron BJ, Thiene G. Arrhythmogenic right ventricular
> cardiomyopathy causing sudden cardiac death in boxer dogs: a new animal
> model of human disease. Circulation. 2004; 109: 1180-1185.). This model
> could be helpful to grasp the pathogenic mechanisms of ARVC/D;
>
> 3) GENETICALLY MODIFIED MICE. (Asano Y, Takashima S, Asakura M, et
> al. Lamr1 functional retroposon causes right ventricular dysplasia in mice.
> Nat Genet. 2004; 36:123-130.).
>
> The possible causes and mechanism include:
>
> (1) APOPTOSIS;
>
> (2) INFLAMMATION;
>
> (3) DYSPLASIA;
>
> (4) TRANSDIFFERENTIATION (Fontaine G, Mallat Z, Fornes P, et al.
> Etiopathogenesis of arrhythmogenic right ventricular dysplasia Ann Cardiol
> Angeiol (Paris). 2000; 49:37-47.) (Wang QW, Liu L. Arrhythmogenic right
> ventricular cardiomyopathy Fa Yi Xue Za Zhi. 2004; 20:35-36.), and
>
> (5) TRANSCRIPTIONAL REGULATION (Asano Y, Takashima S, Asakura M, et al.
> Lamr1 functional retroposon causes right ventricular dysplasia in mice. Nat
> Genet. 2004; 36:123-130.).
>
> Best Regard
>
> Andrés Ricardo Pérez Riera
>
> ----------------------------------------------------------
>
> Português
>
> Prezado Dr Carlos H. Lightowler devido a escassez modelos animais
> existentes a etiologia e patogênese da DAVD permanece desconhecida ou
> incompletamente esclarecida. O mecanismo patofisiológicos não tem sido
> esclarecido pela pouca divulgação do modelo animal. Até o presente momento
> três animais têm sido empregados com este propósito:
>
> 1) GATO DOMÉSTICO: Foxe e col. identificaram neste animal
> cardiomiopatia clínica relevante. Esta cardiomiopatia é muito semelhante e
> lembra muito a DAVD humana. A relevância de este unico modelo felino da
> doença huana será importante na definição da patogênese e na pesquiza de
> mecanismos implicados na progressão da doença (Fox PR, Maron BJ, Basso C,
> Liu SK, Thiene G. Spontaneously occurring arrhythmogenic right ventricular
> cardiomyopathy in the domestic cat: A new animal model similar to the human
> disease. Circulation. 2000; 102:1863-1870..)
> Recentemente Harvey et al. apresentou 2 gatos afetados, No Holter de 24h e
> ECG detectou bloqueio AV completo extrasistoles polimórficas em ambos os
> gatose TV bigeminsimo ventricular e fenômeno R sobre T em um dos gatos
> (Harvey AM, Battersby IA, Faena M, Fews D, Darke PG, Ferasin
> L.Arrhythmogenic right ventricular cardiomyopathy in two cats. J Small Anim
> Pract. 2005; 46:151-156.). Um dos gato teve eposôdio de síncope. No
> Ecocardiograma o VD e AD estavam massivamente dilatados, o VD estava
> hipocinético. A sobrevida dos gatos foi de 3 dias e 16 após o diagnóstico.
> Histopatologicamente um caso revelou Infiltração fibro-gordurosa
> predominantemente no VD na sua parede livre.
>
> 2) CÃES BOXERS: Este parece ser o modelo animal ideal para o estudo
> da enfermidade humana uma vez que se transmite geneticamente, possui a
> mesma etiopatogenia histopatologia, anatomopatologia e clínica dos humanos
> (Basso C, Fox PR, Meurs KM, Towbin JA, Spier AW, Calabrese F, Maron BJ,
> Thiene G. Arrhythmogenic right ventricular cardiomyopathy causing sudden
> cardiac death in boxer dogs: a new animal model of human disease.
> Circulation. 2004; 109: 1180-1185.). Este modelo poderá ahudar a entender a
> patogenia da DAVD.
>
> 3) RATOS GENETICAMENTE MODIFICADOS (Asano Y, Takashima S, Asakura M,
> Shintani Y, Liao Y, Minamino T, Asanuma H, Sanada S, Kim J, Ogai A,
> Fukushima T, Oikawa Y, Okazaki Y, Kaneda Y, Sato M, Miyazaki J, Kitamura S,
> Tomoike H, Kitakaze M, Hori M. Lamr1 functional retroposon causes right
> ventricular dysplasia in mice. Nat Genet. 2004; 36:123-130.).
>
> As possíveis causas e mecanismos incluem:
>
> 1) APOPTOSE;
>
> 2) INFLAMAÇÃO;
>
> 3) DISPLASIA;
>
> 4) TRANSDIFERENCIAÇÃO.
>
> 5) REGULAÇÃO TRANSCRIPCIONAL.
>
> Saudações
>
> Andrés Ricardo Pérez Riera.
>
> ---------- Mensaje reenviado ----------
>
> English - Spanish
>
> Dear friends,
> Let me send this comment to the Forum, which I think may be interesting. I
> am a veterinarian and Professor of Veterinarian Cardiology at the Faculty
> of Veterinary Sciences of the Universidad de Buenos Aires.
> In a breed of our canine patients, the Boxer, there is a disease called
> arrhythmogenic right ventricular cardiomyopathy, which presents outstanding
> similarities to the humane ARVD. Its presents most frequently at around two
> years old (equivalent to about 20 years old in a man).
> The process sometimes evolves without symptoms, except for the presence of
> right ventricular extrasystoles or the consultation is made because of the
> development of "syncopes", most of the times associated to long ventricular
> paroxysmal tachycardias (I attach a tracing, typical of the disease).
>
> http://www.arvd-symposium.org/files/CVDA.jpg
>
> Generally, asymptomatic patients, but with presence of right ventricular
> extrasystoles, are studied with 24 hours Holter. If they present
> ventricular extrasystoles more frequently than 20 per hour, they are
> treated with beta-blockers (generally Atenolol), and if there is some run
> of paroxysmal ventricular tachycardia, mexiletine is added. In general,
> early treatment delays the appearance of ventricular dysfunction, which in
> all cases comes to the end of the process (some four years, if the patient
> is on
> medication). First, right ventricular dysfunction appears (% FA lower than
> 28% and Tei index of >0.40), and then it becomes global cardiac dysfunction
> and death. Sudden deaths are not uncommon (generally, undiagnosed or
> untreated patients).
> There are not many references about the anatomopathologic aspects of the
> disease, though the replacement of the work muscle with fiber tissue has
> been mentioned. There is a clear hereditary predisposition. However,
> studies about it are incomplete.
> Another interesting data. In a series of 57 cases of the disease, all the
> diseased people were associated to a certain type of hair: striped dark
> brown (the breed has various types of hair: light brown, light brown with
> white chest, light brown with white neck, and the mentioned striped dark
> brown). Some possible resemblance to the Naxus illness?
> I hope these comments are useful, at least as an anecdote. However, this
> breed could be an excellent experimental natural model for the study of
> this disease.
> Best regards for all,
>
> Carlos H. Lightowler DVM
>
> --------------------------------------------------
>
> Estimados amigos
> Me permito enviar al foro este comentario porque creo que puede
> ser de interés. Soy médico veterinario y Profesor de Cardiología
> Veterinaria en la Facultad de Ciencias veterinarias de la Universidad de
> Buenos Aires. En una raza de nuestros pacientes caninos, la Bóxer existe
> una enfermedad denominada Cardiomiopatía ventricular derecha arritmogénica
> que presenta llamativas similitudes con la AVRD del hombre. Su presentación
> más frecuente es alrededor de los 2 años de edad (equivalente a unos 20
> años del hombre). El proceso cursa a veces asintomáticamente, salvo por la
> presencia de extrasístoles ventriculares derechas o se realiza la consulta
> por el desarrollo de "síncopes", la mayoría de las veces asociados a
> taquicardias paroxísticas ventriculares prolongadas (adjunto un trazado,
> típico de la enfermedad).
>
> http://www.arvd-symposium.org/files/CVDA.jpg
>
> Generalmente los pacientes asintomático pero con presencia de
> extrasístoles ventriculares derechas son estudiados con Holter de 24 hs. Si
> presentan extrasístoles ventriculares con mayor frecuencia de 20 por hora,
> son tratados con betabloqueantes (generalmente Atenolol) y si existe alguna
> corrida de taquicardia ventricular paroxística, se agrega mexiletina. En
> general el tratamiento precoz, retrasa la aparición de la disfunción
> ventricular, la cual en todos los casos llega hacia el final del proceso
> (unos cuatro años si el paciente está medicado). Primero aparece disfunción
> ventricular derecha (%AF menor de 28% e índice de Tei de >0,40), para luego
> convertirse en disfunción cardiaca global y muerte. No son raras las
> muertes súbitas (generalmente en los pacientes no diagnosticados o no
> tratados). No existen demasiadas referencias respecto a los aspectos
> anatomopatológicos de la enfermedad, si bien se ha mencionado en algunos
> casos el reemplazo del músculo de trabajo por tejido fibroso. Existe una
> clara predisposición hereditaria. Sin embargo los estudios al respecto son
> incompletos.
> Otro dato interesante. En una serie de 57 casos de la
> enfermedad, todos los enfermos estaban asociados a un determinado pelaje:
> marrón oscuro atigrado (la raza posee varios pelajes: marrón claro, marrón
> claro con pecho blanco, marrón claro con cuello blanco y el mencionado
> marrón oscuro atigrado). Alguna posible semejanza con la enfermedad de
> Naxos? .
> Espero que estos comentarios sirvan, aunque se como anécdota.
> Sin embargo, esta raza puede ser un excelente modelo experimental natural
> para el estudio de la enfermedad.
> Afectuosos saludos para todos
>
> Carlos H. Lightowler DVM
--
Dr. Sergio Dubner
Director
Dr. Edgardo Schapachnik
Director
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