[ARVD-FORUM] New papers commentaries. Dr. Perez Riera
ARVD Symposium
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Mon Jul 25 19:49:17 ART 2005
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NEW PAPERS COMMENTARIES
Dear Edgardo: interesting cases report from Kerala India. Please send to
Brugada and ARVC/D Virtual Symposium
Andrés.
Harikrishnan S, Kumar Dora S, Tharakan JM. Brugada syndrome.Int J Cardiol.
2005 Jul 20;102(3):539-541.
Sree Chitra Tirunal Institute for Medical Sciences and Technology,
Thiruvananthapuram-695004, Kerala, India.
Two siblings with features of Brugada syndrome are reported. One of them had
permanent pacemaker implantation elsewhere where he was evaluated for
recurrent syncope and diagnosed to have tri-fascicular block. He continued
to have syncopal episodes and subsequently detected to have runs of
polymorphic ventricular tachycardia picked up on a routine ECG. His sibling
also was found to have features of Brugada syndrome.
Commentaries: The term trifascicular block is used to designate cases of
association CRBBB + Left Anterior Fascicular Block (LAFB) or Left
Posterior Fascicular Block (LPFB) and first-degree AV block (incomplete AV
block: Long PR interval) because the AV block may be sited in the AV node,
the bundle of His or left bundle branch proximally. In BrS the PR interval
of ECG is prolonged in approximately 50% of the cases, additionally CRBBB is
frequently and characteristic and in approximately 9.5% of cases marked
left QRS axis deviation is observed according to a prospective three-year
follow-up study between a population of workers in the Tokyo area(1). In
some cases, this marked left QRS axis deviation in frontal plane is
secondary to LAFB. Then, the presence of trifascicular block is
theoretically possible in the BrS: association of prolonged PR interval +
CRBBB + LAFB. I believe that this is the first time that one reveals in
literature trifascicular block in the BrS.
1) Atarashi H, Ogawa S, Harumi K, et al. Idiopathic Ventricular
Fibrillation Investigators. Three-year follow-up of patients with right
bundle branch block and ST segment elevation in the right precordial leads:
Japanese Registry of Brugada Syndrome. Idiopathic Ventricular Fibrillation
Investigators. J Am Coll Cardiol 2001; 37:1916-1920.
Second paper commentaries
Thiene G, Basso C, Calabrese F, Angelini A, Valente M. Twenty years of
progress and beckoning frontiers in cardiovascular pathology
Cardiomyopathies. Cardiovasc Pathol. 2005 Jul-Aug; 14(4):165-169
Institute of Pathological Anatomy, University of Padua Medical School,
Padua, Italy.
In the last 20 years, with the advent of cardiac transplantation and the
availability of molecular biology techniques, major advancements were
achieved in the understanding of cardiomyopathies. Novel cardiomyopathies
have been discovered (arrhythmogenic right ventricular, primary restrictive,
and noncompacted myocardium) and added in the update of WHO classification.
Myocarditis was also included with the name "inflammatory cardiomyopathy."
Adenoviruses and parvoviruses were found to be frequent cardiotropic viruses
in addition to enteroviruses. The extraordinary progress accomplished in
molecular genetics of inherited cardiomyopathies allowed to establish
hypertrophic and restrictive cardiomyopathies as sarcomeric ("force
generation") diseases, dilated cardiomyopathies as cytoskeleton ("force
transmission") disease, and arrhythmogenic right ventricular cardiomyopathy
(ARVC) as cell junction disease. If we consider also cardiomyopathy as ion
channel disease (long and short QT syndrome, Brugada syndrome, and
cathecolaminergic polymorphic ventricular tachycardia), because they are
diseases of the myocardium associated with electrical dysfunction, then a
genomic/postgenomic classification of inherited cardiomyopathies may be put
forward: cytoskeletal cardiomyopathy, sarcomeric cardiomyopathy, cell
junction cardiamyopathy and ion channel cardiomyopathy.
PMID: 16009312 [PubMed - in process]
What the forum think about this new classification of cardiomyopathies?
Best Regard
Andres.
CARDIOMYOPATHIES CLASSIFICATION
(1.1) - Dilated (Congestive) CardioMyopathy (DCM): It is
considered as cytoskeleton ("force transmission") disease.
(1.2) - Hypertrophic CardioMyopathy (HCM)
(1.3) - Restrictive CardioMyopathy (RCM)
Observation: HCM and RCM are considered as sarcomeric ("force
generation") diseases;
(1.4) - Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia
(ARVC/D): It is considered as cell junction disease;
(1.5) - Unclassified: It includes a few cases that do not fit
readily into any group (e.g,fibroelastosis, non-compacted myocardium, and
systolic dysfunction with minimal dilatation and mitochondrial disease).
(1.6) Myocarditis was also included with the name "inflammatory
cardiomyopathy."
(1.7) Ion channel cardiomyopathies1: If we consider
also cardiomyopathy as ion channel disease (long and short QT syndrome,
Brugada syndrome, and cathecolaminergic polymorphic ventricular
tachycardia), because they are diseases of the myocardium associated with
electrical dysfunction.
1) Thiene G, Basso C, Calabrese F, Angelini A, Valente M. Twenty years of
progress and beckoning frontiers in cardiovascular pathology
Cardiomyopathies. Cardiovasc Pathol. 2005 Jul-Aug; 14(4):165-169
-----------------------------------------
Harikrishnan S, Kumar Dora S, Tharakan JM. Brugada syndrome.Int J Cardiol.
2005 Jul 20;102(3):539-541.
Sree Chitra Tirunal Institute for Medical Sciences and Technology,
Thiruvananthapuram-695004, Kerala, India.
Se informo sobre dos hermanos con caracteristicas de sindrome de Brugada.
Uno de ellos tenia marcapasos permanente implantado en otro sitio que en el
que se lo evaluo por sincope recurrente y se lo diagnostico con bloqueo
trifascicular. Continuo presentando episodios sincopales y a continuacion se
detectaron rafagas de taquicardia ventricular polimorfica en un ECG de
rutina. En su hermano tambien se hallaron caracteristicas de sindrome de
Brugada.
Comentarios: el termino bloqueo trifascicular se emplea para designar casos
de asociacion de BCRD + bloqueo divisional antero-superior izquierdo (BDASI)
o bloqueo divisional postero-inferior izquierdo (BDPII) y bloqueo AV de
primer grado (bloqueo AV incompleto: intervalo PR prolongado) porque el
bloqueo AV puede estar ubicado en el nodo AV, el haz de His o rama izquierda
proximalmente. En el SBr el intervalo PR del ECG se encuentra prolongado en
aproximadamente el 50% de los casos, ademas el BCRD es frecuente y
caracteristico y en aproximadamente 9,5% de los casos, se observa eje QRS
marcadamente desviado hacia la izquierda segun el estudio prospectivo de
seguimiento de tres anios entre una poblacion de empleados del area de Tokio
(1). En algunos casos, esta desviacion marcada hacia la izquierda del eje
QRS en el plano frontal es secundaria a BDASI. Luego, la presencia de
bloqueo trifascicular es teoricamente posible en el SBr: asociacion de
intervalo PR prolongado + BCRD + BDASI. Creo que esta es la primera vez que
se revela en la literatura bloqueo trifascicular en el SBr.
1) Atarashi H, Ogawa S, Harumi K, et al. Idiopathic Ventricular
Fibrillation Investigators. Three-year follow-up of patients with right
bundle branch block and ST segment elevation in the right precordial leads:
Japanese Registry of Brugada Syndrome. Idiopathic Ventricular Fibrillation
Investigators. J Am Coll Cardiol 2001; 37:1916-1920.
Comentarios del segundo trabajo:
Thiene G, Basso C, Calabrese F, Angelini A, Valente M. Twenty years of
progress and beckoning frontiers in cardiovascular pathology
Cardiomyopathies. Cardiovasc Pathol. 2005 Jul-Aug; 14(4):165-169
Institute of Pathological Anatomy, University of Padua Medical School,
Padua, Italy.
En los ultimos 20 anios, con el advenimiento del transplante cardiaco y la
disponibilidad de las tecnicas de biologia molecular, se lograron
importantes avances en la comprension de la miocardiopatias. Las nuevas
miocardiopatias se han descubierto (arritmogenica del ventriculo derecho,
restrictiva primaria, miocardio no compacto) y agregado en la actualizacion
de la clasificacion de la OMS. La miocarditis tambien se incluyo con el
nombre de "miocardiopatia inflamatoria". Se encontro que los adenovirus y
parvovirus son virus cardiotropicos frecuentes ademas de los enterovirus. El
progreso extraordinario logrado en la genetica molecular de las
miocardiapatias hereditarias permitio establecer las miocardiopatias
hipetroficas y restrictivas como enfermedades sarcomericas ("generacion de
fuerzas"), miocardiopatias dilatadas como la enfermedad del citoesqueleto
("transmision de fuerzas") y la miocardiopatia arritmogenica del ventriculo
derecho (M/DAVD) como una enfermedad de la union celular. Si consideramos
tambien la miocardiopatia como una enfermedad del canal ionico (sindromes de
QT prolongado y corto, sindrome de Brugada y la taquicardia ventricular
polimorfica catecolaminergica), puesto que son enfermedades del miocardio
asociadas con disfuncion electrica, luego podria presentarse una
clasificacion genomica/postgenomica de las miocardiopatias hereditarias: la
miocardiopatia del citoesqueleto, miocardiopatia sarcomerica, miocardiopatia
de la union celular y miocardiopatia del canal ionico.
PMID: 16009312 [PubMed - in process]
Que piensa el foro sobre esta nueva clasificacion de las miocardiopatias?
Saludos cordiales,
Andres
CLASIFICACION DE LAS MIOCARDIOPATIAS
(1.1) - Miocardiopatia dilatada (congestiva): considerada como enfermedad
del citoesqueleto ("transmision de fuerza")
(1.2) - Miocardiopatia hipertrofica;
(1.3) - Miocardiopatia restrictiva;
Observacion: la miocardiopatia hipertrofica y la restrictiva son
consideradas como enfermedades sarcomericas ("generacion de fuerzas");
(1.4) - Miocardiopatia/displasia arritmogenica del ventriculo derecho
(M/DAVD): considerada como enfermedad de la union celular;
(1.5) - Sin clasificar: incluye unos pocos casos que no encajan facilmente
en cualquier grupo (por ej.: fibroelastosis, miocardio no compacto y
disfuncion sistolica con dilatacion minima y enfermedad mitocondrial);
(1.6) - La miocarditis tambien se incluyo con el nombre de "miocardiopatia
inflamatoria";
(1.7) - Miocardiopatias del canal ionico (1): si consideramos tambien la
miocardiopatia como enfermedad del canal ionico (sindromes de QT prolongado
y corto, sindrome de Brugada y taquicardia ventricular polimorfica
catecolaminergica), porque son enfermedades del miocardio asociados con
disfuncion electrica.
1) Thiene G, Basso C, Calabrese F, Angelini A, Valente M. Twenty years of
progress and beckoning frontiers in cardiovascular pathology
Cardiomyopathies. Cardiovasc Pathol. 2005 Jul-Aug; 14(4):165-169
--
Dr. Sergio Dubner
Director
Dr. Edgardo Schapachnik
Director
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