[SCD-FORUM] WATCHING THE WEBCAST BY DR. FRANK MARCUS. THREE EXPERT QUESTIONS BY DR. KATARZYNA WLODARSKA

[SCD Symposium]

Right Ventricular Dysplasia/Cardiomyopathy
Frank Marcus

Dr. Katarzyna Wlordaska (Poland)
- Is ARVC/D a dysplasia? From the patophysiological point of view the  
term "dysplasia" should be reserved for Uhl's anomaly, which in my  
opinion should not be included to "right ventricular  
cardiomyopathy",  even in broad meaning. Am I right?
Dr. Frank Marcus (U.S.A.)

- I will attempt to answer the questions posed by Dr.Wlodarska.

Historically, the term right ventricular dysplasia was proposed by  
Dr. Fontaine and colleagues when they first described the disease in  
1977, and this term has persisted.  Dr. Fontaine  maintaines that the  
term "dysplasia" is appropriate since it is defined as "a trouble of  
development".



Dr. Katarzyna Wlordaska (Poland)

- Do you think "Arrhythmogenic right ventricular cardiomyopathy/ 
dysplasia" is a synonyme of the right ventricular cardiomyopathy?  If  
not, where is its place in the new Classification of Cardiomyopathies  
according to AHA Scientific Statement (Circulation, 2006,113,1807-1816)?

Dr. Frank Marcus (U.S.A.)
- This disease fits the classification proposed in the article  
"Contemporary Definitions and Classification of the Cardiomyopathies"  
by Maron B.J. et al in Circulation 2006;113:1807-1816.  Their  
proposed definition of a cardiomyopathy was"Cardiomyopathies are a  
heterogeneous gruop of diseases of the myocardium associated with  
mechanical and/or electrical dysfunction that usually (but not  
invariably) exhibit inappropriate ventricular hypertrophy or  
dilatation and are due to a variety of causes that frequently are  
genetic"    It would be well if those of us who are involved in the  
study of this entity agree on a terminology.  This may be a point of  
discussion at a proposed conference to reasses the Task Force  
Criteria for the diagnosis of this disease.  Until then we are left  
with ARVC, ARVD,  ARVC/D or ARVD/C.


Dr. Katarzyna Wlordaska (Poland)
- I absolutely share your opinion on low diagnostic value of MRI in  
ARVC/D. I am afraid that overestimation of its value and lack of  
knowledge about the essential role of ECG  is very common. However,  
ECG changes depend on extensiveness of the disease. What is a  
diagnostic value of ECG in borderline cases?  Does ECG differentiate  
ARVC/D from Uhl's anomaly?

Dr. Frank Marcus (U.S.A.)
- You raise the question of the diagnostic value of the ECG in  
borderline cases.  I think the ECG is exremely valuable under these  
circumstances, particularly T wave inversion beyond V2..  When I am  
presented with a patient in whom the differential diagnosis is  
idiopathic (RVOT) tachycardia, and the ECG fits the above  
description, it greatly heightens my suspicion that the patient has  
ARVC/D.  T wave inversion in V1-V3 is present in less than 3% of  
apparently healthy subjects who are 19-45 years of age but is found  
in well over 50% of patients with ARVC/D. Ref.  Marcus F. Am. J.  
Cardiol 2005;95;1070-1071.  We are currently evaluating the extensive  
information from the North American ARVC/D database to further study  
the relation of the ECG changes with the extent of right ventricular  
involvement.   Uhls anomaly is so rare that it does not enter into my  
differential diagnosis of ARVC/D.

--
Dr. Sergio Dubner
President of Scientific Committee

Dr. Edgardo Schapachnik
President of Steering Committee