[ARVD-FORUM] Heart transplatation Dr. Martini

Thu Jun 23 22:59:10 ART 2005

English - Spanish

Dear Andres,
I am sorry You did not appreciate the birth of the Janus syndrome. Probably
Sancho Panza would have done this.
I appreciate Your definition of "The Brugada syndrome is a congenital
syndrome displaying an autosomal dominant mode of transmission in patients
with a probably structurally normal heart". So You little by little admit
that the popular theory of functional disease can be wrong.
I also appreciate " I think that is necessary more evidences. The true is
not near us until to day..I think."
This was what I have been saying and published in the last years (More
evidence-based data are required for a consensus on the so called Brugada
syndrome: Martini et al. European Heart Journal 2003).
You agree that it is necessary more evidence. Can You honestly tell us why
not A SINGLE CASE OF DETAILED NORMAL NECROPSY have been presented yet to the
Medical community in any paper. You know very well all the literature.
Can You provide a SINGLE REFERENCE? I have asked You this question several
times but I never had an answer. Why?
Differently from this, so less than 50 hearts, have been found to have
organic heart disease at necropsy, INCLUDED 17 OUT OF 18 HEARTS EXAMINED BY
Kirshner: this is not INCIDENTALLY, as you say, BUT A RULE!
Please stop to represent the theory that a functional heart disease can
induce hibernating myocardium and fibrosis. This is science-fiction and not
evidence based medicine. You cannot compare the effects of a permanent
atrial fibrillation with the effects of a strange ECG.
bortolo

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

Estimado Andres:
Que lastima que Ud. no apreciara el nacimiento del sindrome de Jano.
Probablemente Sancho Panza lo hubiera hecho.
Le agradezco su definicion de: "El sindrome de Brugada es un sindrome
congenito que presenta un modo dominante autosomico de transmision en
pacientes con un corazon probablemente estructuralmente normal". Asi que
poco a poco admite que la teoria popular de la enfermedad funcional puede
estar equivocada. Ademas le agradezco: "Creo que mas evidencias son
necesarias. La verdad no estuvo cerca hasta hoy...creo".
Esto es lo que estuve diciendo y publicando estos ultimos anios (More
evidence-based data are required for a consensus on the so called Brugada
syndrome: Martini et al. European Heart Journal 2003).
Ud. esta de acuerdo con que mas evidencia es necesaria. Puede decirme
honestamente por que ni UN SOLO CASO DE NECROPSIA NORMAL DETALLADA se ha
presentado aun a la comunidad medica en ningun articulo? Ud. conoce muy bien
toda la literatura. Puede darme UNA SOLA REFERENCIA? Le he hecho esta
pregunta varias veces pero nunca obtuve una respuesta. Por que?
A diferencia de esto, se han encontrado no menos de 50 corazones con
cardiopatia organica en la necropsia, INCLUYENDO 17 DE 18 CORAZONES
EXAMINADOS POR Kirshner: esto no es ACCIDENTAL, como Ud. dice, SINO LA
REGLA!
Por favor, deje de representar la teoria de que una cardiopatia funcional
puede inducir miocardio hibernado y fibrosis. Esto es ciencia ficcion y no
medicina basada en la evidencia. No puede comparar los efectos de una
fibrilacion auricular permanente con los efectos de un ECG inusual.
bortolo

-----Messaggio originale-----
Da: arvd-forum-bounces at ishne.org [mailto:arvd-forum-bounces at ishne.org] Per
conto di ARVD Symposium
Inviato: sabato 11 giugno 2005 20.59
A: arvd-forum at arvd-symposium.org
Oggetto: [ARVD-FORUM] Heart transplatation Dr. Perez Riera

Forum of the First International Symposium on Arrhythmogenic Right
Ventricular Dysplasia on Internet  |  Foro del Primer Simposio Internacional
de Displasia Arritmogénica de Ventrículo Derecho en Internet  |  Forum do
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_______________________________________
English - Spanish

Dear Dr Martini: The Brugada syndrome is a congenital syndrome displaying an
autosomal dominant mode of transmission in patients with a probably
structurally normal heart. Sudden unexplained nocturnal death syndrome
(SUNDS, also known as SUDS) and BrS have been shown to be phenotypically,
genetically, and functionally the same disorder.  The disease has been
linked to mutations in SCN5A, a gene located on the short arm of chromosome
3 (p21-24) that encodes for the alpha subunit of the sodium channel.  The
syndrome is characterized by a dynamic ST segment elevation (accentuated J
wave) in the leads V1 to V3 of the ECG followed by negative T wave.
Incomplete or complete Right bundle branch block (IRBBB or CRBBB) of varying
degrees is observed in some patients. The syndrome is associated with
syncope and a relatively high incidence of sudden cardiac death secondary to
the development of polymorphic ventricular tachycardia that may degenerate
into ventricular fibrillation. An acquired form of the Brugada syndrome is
also recognized, caused by a wide variety of drugs and conditions that alter
the balance of currents active during the early phases of the action
potential.  Among patients with Arrhythmogenic Right Ventricular
Cardiomyopathy/Dysplasia (ARVC/D), there is a subpopulation with a clinical
and electrocardiographic pattern very similar to that of the BrS.  These
cases of ARVC/D are thought to represent an early or concealed form of the
disease.

A link or overlap between BrS and ARVC/D may exist when:

1)       Documented Polymorphic Ventricular Tachycardia / Ventricular
fibrillation associated with a type 1 ST segment elevation in V1 to V3 with
a positive family history for Sudden Cardiac Death in a first-degree
relative younger than 45 years old;

2)       Syncope or nocturnal agonal respiration;

3)        Endomyocardial biopsy from RV free wall, Right Ventricular Inflow
Tract, RVOT, and apical region (dysplasia triangle) reveals microscopic
lesions compatible with ARVC/D: fatty or fibro-fatty substitution.

The three main elements of the initial description of BrS (Right Bundle
Branch Block, persistent ST segment elevation and normal QT interval) today
cannot be included in a current definition of the syndrome because:

1)       RBBB: We demonstrate by VCG that there are cases without IRBBB or
CRBBB that explain the non-existence of final S broad wave in the left
leads. Cases with only the ST segment elevation;

2)       The ST segment elevation is not persistent (It is dynamic);

3)       The QT interval is not normal because frequently there is a minimal
QT interval prolongation, especially from V1 to V3 like ARVD.

4) Organic? Electrical or fuctional disease????

BrS could begin as a primary electrical or functional disease without
organic substrate and in evolution would chronically and possibly develop
structural alterations, as it is observed in electrical remodeling in atrial
fibrillation and in ventricular hibernation (Ausma J, Wijffels M, Vaneys G,
et al. Differentiation of atrial cardiomyocytes as a result of chronic
atrial fibrillation. Am J Pathol. 1997; 151:985-97.)

Fibrosis was observed after transplantation.

Occasionally some structural abnormalities were found in the conduction
system, in Southeast Asian refugees( Kirschner RH, Eckner FAA, Baron RC: The
cardiac pathology of   Sudden Unexplained Nocturnal Death in Southeast Asian
refugees. JAMA 1986; 256:2700.).

Dear Bortolo I think that is necessary more evidences. The true is not near
us until to day..I think.

Best

Andres.

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

Estimado Dr. Martini:
El sindrome de Brugada es un sindrome congenito que presenta un modo
dominante autosomico de transmision en pacientes con un corazon
probablemente estructuralmente normal. Se ha demostrado que el sindrome de
muerte nocturna inexplicable e inesperada (SUNDS o SUDS por sus siglas en
ingles) y el SBr son fenotipica, genetica y funcionalmente el mismo
trastorno. La enfermedad se ha vinculado con las mutaciones en el SCN5A, un
gen localizado en el brazo corto del cromosoma 3(p21-24) que codifica la
subunidad alfa del canal de sodio. El sindrome se caracteriza por
supradesnivel dinamico del segmento ST (onda J acentuada) en las
derivaciones V1 a V3 del ECG, seguido por onda T negativa.
El bloqueo completo o incompleto de la rama derecha (BCRD o BIRD) de grados
variables se observa en algunos pacientes. El sindrome esta asociado con
sincope y una incidencia relativamente alta de muerte subita secundaria al
desarrollo de taquicardia ventricular polimorfica que puede degenerar en
fibrilacion ventricular. Una forma adquirida del sindrome de Brugada tambien
se reconoce, causada por una gran variedad de drogas y condiciones que
alteran el equilibrio de corrientes activas durante las etapas precoces del
potencial de accion. Entre los pacientes con Miocardiopatia/Displasia
Arritmogenica del Ventriculo Derecho (M/DAVD) hay una subpoblacion con un
patron clinico y electrocardiografico muy similar al del SBr. Se piensa que
estos casos de M/DAVD representan una forma precoz u oculta de la
enfermedad.

Podria existir un vinculo o superposicion entre el SBr y la M/DAVD si
hubiera:

1)	Taquicardia ventricular polimorfica/fibrilacion ventricular
documentada
en asociacion con supradesnivel del segmento ST tipo 1 en V1 a V3 con una
historia familiar positiva de muerte subita en un familiar en primer grado
menor de 45 anios;

2)	Sincope o respiracion agonica nocturna;

3)	Biopsia endomiocardica de la pared libre del VD, tracto de entrada
del
ventriculo derecho, TSVD y region apical (triangulo de la displasia) que
revelen lesiones microscopicas compatibles con M/DAVD: sustitucion adiposa o
fibroadiposa.

Los tres elementos principales de la descripcion inicial del SBr (bloqueo de
rama derecha, supradesnivel persistente del segmento ST e intervalo QT
normal), hoy no podrian ser incluidos en una definicion actual del sindrome
porque:

1)	BRD: demostramos por VCG que hay casos sin BIRD o BCRD, lo que
explica la
no existencia de onda S final y ancha en las derivaciones izquierdas. Casos
con supradesnivel del segmento ST unicamente;

2)	El supradesnivel del segmento ST no es persistente (es dinamico);

3)	El intervalo QT no es normal porque con frecuencia hay una minima
prolongacion del intervalo QT, especialmente de V1 a V3, como en la DAVD.

4)	Enfermedad organica? Electrica o funcional????

El SBr puede comenzar como una enfermedad electrica primaria o funcional sin
un sustrato organico y en su evolucion podria desarrollar cronicamente y
posiblemente alteraciones estructurales, como se observa en el remodelado
electrico en la fibrilacion auricular y la hibernacion ventricular (Ausma J,
Wijffels M, Vaneys G, et al. Differentiation of atrial cardiomyocytes as a
result of chronic atrial fibrillation. Am J Pathol. 1997; 151:985-97).

Ocasionalmente algunas anormalidades estructurales se encontraron en el
sistema de conduccion en refugiados del Sudeste asiatico (Kirschner RH,
Eckner FAA, Baron RC: The cardiac pathology of   Sudden Unexplained
Nocturnal Death in Southeast Asian refugees. JAMA 1986; 256:2700).

Estimado Bortolo, creo que es necesario tener mas evidencias. La verdad no
estaba cerca de nosotros hasta este dia... creo.

Saludos,

Andres