[ARVD-FORUM] EXPERTS ASK, EXPERTS ANSWER. Dr. Perez Riera

[ARVD Symposium]

English - Portuguese

Dear Dr Katarzyna Wlodarska,
Noninvasive detection of RV myocardial fibro-fatty changes in ARVD/C is
possible by MDE-MRI. MRI findings had an excellent correlation with
histopathology and predicted inducible VT on PES, suggesting a possible role
in evaluation and diagnosis of patients with suspected ARVD/C.Tandri H,
Saranathan M, Rodriguez ER,  et al.Noninvasive detection of myocardial
fibrosis in arrhythmogenic right ventricular cardiomyopathy using
delayed-enhancement magnetic resonance imaging. J Am Coll Cardiol.
2005;45:98-103.)MRI must be performed using cardiac gating, and should
include both cine-MR sequences for evaluation of segmental and global RV
function or any morphological change of the RV shape, and anatomic sequences
to detect fatty or fibro-fatty infiltration of the RV myocardium.(Jacquier
A, Bressollette E, Laissy JP, et al. MR imaging and arrhythmogenic right
ventricular dysplasia (ARVD) J Radiol. 2004;85:721-724.) There are an high
frequency of "misdiagnosis" of ARVD/C is due to over-reliance on the
presence of intramyocardial fat/wall thinning on MRI, incomplete diagnostic
testing, and lack of awareness of the Task Force criteria. Diagnosis of
ARVD/C cannot rely solely upon qualitative features on MRI. Bomma C, Rutberg
J, Tandri H, et al.Misdiagnosis of arrhythmogenic right ventricular
dysplasia/cardiomyopathy.J Cardiovasc Electrophysiol. 2004;15:300-306. EMB
appears the most reliable diagnostic technique, with significant prognostic
and therapeutic implications.The LV may also be involved by the same disease
process. Moreover, inflammation can be superimposed on ARVC/D, resulting in
a wide spectrum of clinical presentation which can mimick idiopathic dilated
cardiomyopathy. In this cases is imperative EMB (Fontaine G, Hebert JL,
Prost-Squarcioni C, et al Arrhythmogenic right ventricular dysplasiaArch Mal
Coeur Vaiss. 2004;97: 1155-1159.)

In sarcoidosis sometimes is necessary EMB or autopsy for diagnostic
distinction. The differential diagnosis is very important because the
treatment with corticosteroids may benefit those with sarcoidosis but is not
expected to be useful in cases with ARVD or ARVC.Ott P, Marcus FI, Sobonya
RE, et al. Cardiac sarcoidosis masquerading as right ventricular
dysplasia.Pacing Clin Electrophysiol. 2003 ;26:1498-503.

Best Regards

Andrés Ricardo Pérez Riera

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Portugues

Prezado Dr Katarzyna Wlodarska,
Detecção não invasiva das mudanças fibrogordurosas do miocárdio na DAVD é
possível utilizando MDE-RNM. Os achados da RNM apresenta excelente
correlação com a histopatologia e possui valor preditivo de inductibilidade
no estudo eletrofisiológico, assinalando que este método não invasivo possui
um importante papel na avaliação dos pacientes suspeitos d seram portadores
de DAVD.Tandri H, Saranathan M, Rodriguez ER,  et al.Noninvasive detection
of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy
using delayed-enhancement magnetic resonance imaging. J Am Coll Cardiol.
2005;45:98-103.)

A RNM deve ser realizada utilizando "gating"cardíaco e deveria incluir a
CINE RM  seqüencial com o intuito de avaliar a função segmentar e global do
VD e/ou as mudanças morfológicas do aspecto do VD e sequencia anatômica para
detectar infiltração gordurosa ou fibrogordurosa no miocárdio do VD
(Jacquier A, Bressollette E, Laissy JP, et al. MR imaging and arrhythmogenic
right ventricular dysplasia (ARVD). J Radiol. 2004;85:721-724.) Existe uma
lata freqüência de diagnósticos equivocados por sobreestimar a presença de
gordura intra-miocárdica e afinamento da parede na RNM, teste diagnósticos
incompletos e falta de emprego dos critérios da Task Force. O diagnóstico da
DAVD não pode ser realizado apenas pela avaliação quantitativa RNM. Bomma C,
Rutberg J, Tandri H, et al.Misdiagnosis of arrhythmogenic right ventricular
dysplasia/cardiomyopathy.J Cardiovasc Electrophysiol. 2004;15:300-306. A BEM
é a técnica com maior implicância prognostica e terapêutica. O VE pode
também estar acometido pela doença e existir inflamação super-imposta,
resultando em um amplo espectro de apresentação clínica que pode mimetizar
cardiomiopatia dilatada e idiopática neste caso é imperativo na BEM(Fontaine
G, Hebert JL, Prost-Squarcioni C, et al Arrhythmogenic right ventricular
dysplasiaArch Mal Coeur Vaiss. 2004;97: 1155-1159.)

Na sarcoidose miocárdica é necessária a BEM para a eventual distinção
diagnóstica. O diagnóstico é muito importante porque o tratamento com
corticoide beneficia os sarcoidóticos, orem não aos portadores de DAVD.(Ott
P, Marcus FI, Sobonya RE, et al. Cardiac sarcoidosis masquerading as right
ventricular dysplasia.Pacing Clin Electrophysiol. 2003 ;26:1498-503.

Saudações

Andrés Ricardo Pérez Riera

_______________________________________


> English - Spanish
>
> Dear Dr Perez Riera,
> let me desagree with you again. May I ask you some practical questions?
> Could you frankly answer me if you perform biopsy in all your patients with
> ARVC? Even in those with typical changes in ECG, Echo and MRI? Even in
> patients with  well controled arrhythmia without syncope? What about
> patients with paper thin RV wall? How the result of a biopsy influence your
> therapeutical decisions when you don't have any doubts that a patient
> should have ICD inserted?
> Yours sincerely,
>
> Katarzyna Wlodarska
>
> ------------------------
>
> Estimado Dr. Perez Riera:
> Dejeme discrepar con Ud. nuevamente. Podria hacerle algunas preguntas
> practicas? Podria responderme en forma franca si Ud. realiza biopsias en
> todos sus pacientes con DAVD? Incluso en aquellos con cambios tipicos en
> ECG, Eco y IRM? Incluso en pacientes con arritmia bien controlada sin
> sincope? Que pasa con los pacientes con paredes del VD delgadas como un
> papel? Como influye el resultado de una biopsia en sus decisiones
> terapeuticas cuando no tiene ninguna duda de que un paciente deberia tener
> un CDI implantado?
> Atentamente,
>
> Katarzyna Wlodarska
>