Circulation. 1998;97:1532-1535
(Circulation. 1998;97:1532-1535.)
© 1998 American Heart Association, Inc.
Arrhythmogenic Right Ventricular Cardiomyopathies
Clinical Forms and Main Differential Diagnoses
G. Fontaine, MD, PhD;
F. Fontaliran, MD;
; R. Frank, MD
From the Department of Cardiology, Hôpital Jean Rostand, Ivry sur
Seine, France.
Correspondence to Guy Fontaine, MD, PhD, Department of Cardiology, Hôpital Jean Rostand, 39 rue Jean Le Galleu, 94200 Ivry sur Seine, France.
Key Words: Editorials • cardiomyopathy • genetics • apoptosis • death, sudden
In this issue
of Circulation, Burke et al1 report
the results of a morphological and morphometric analysis of RV
myocardium to differentiate fatty infiltration of the RV
free wall from fibrofatty infiltration, which is the
histological marker of ARVC. A similar analysis
was previously presented by Thiene et
al,2 classifying their patients as having
"lipomatous" as opposed to "fibrolipomatous" replacement of the
myocardium and putting the two entities in the same group.
Unless small areas of fibrosis are overlooked, the two subgroups of
Burke et al and Thiene et al have the histological
picture of myocardium intermingled with and/or replaced by
fat without associated fibrosis. In this population of patients who
died suddenly, we are faced with the observation that strands of
myocardial fibers within fat without fibrosis may lead to sudden death.
This possibility has rather important consequences, because a large
proportion of seemingly normal hearts have fatty infiltration of the RV
myocardium.3 It may be that the fatty
pattern in these patients represents the early stage of the
disease before the appearance of fibrotic tissue. Therefore, a large
proportion of normal individuals may in fact have the prerequisite for
the development of RV cardiomyopathies.
It is well known that a large majority of patients with ARVD have
histological evidence suggestive of inflammation. It
may be that if myocarditis is superimposed on the background of
myocardium interspersed by fat, strands of
cardiomyocytes involved in myocarditis will produce
fibrosis and will transform the purely fatty to the fibrofatty form.
. . . [Full Text of this Article]
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