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From the Department of Cardiovascular Pathology, Armed Forces Institute
of Pathology, Washington, DC.
Correspondence to E-mail burke{at}email.afip.osd.mil
Background—The relationship between arrhythmogenic right
ventricular cardiomyopathy (ARVC) and
pure fat replacement of the right ventricle is
unclear.
Methods and Results—Myocardial thickness, epicardial fat
thickness, percent fibrosis, and intramyocardial fat infiltration were
measured in 16 sections each from 25 hearts with typical (fibrofatty)
ARVC, 7 hearts with fat replacement of the right ventricle without
fibrosis (FaRV), and 18 control hearts from patients who died of
noncardiac causes. Patients with fibrofatty ARVC were younger than
those with FaRV (31±14 versus 44±13 years, P=.02), more
likely to have a history of arrhythmias or a family history of
premature sudden death (56% versus 0%, P=.01), more likely
male (80% versus 29%, P=.02), and less likely to have
coexisting conditions that might have predisposed to sudden death (12%
versus 86%, P<.001). Fibrofatty ARVC was characterized by
right ventricular myocardial thinning, fat infiltration of
the anterobasal and posterolateral apical right ventricle,
subepicardial left ventricular fibrofatty replacements
(64%), myocyte atrophy (96%), and lymphocytic myocarditis (80%).
FaRV showed normal or increased myocardial thickness, a diffuse
increase in intramyocardial and epicardial fat, little inflammation,
and an absence of myocardial atrophy. Intramyocardial fat was
frequently seen in normal hearts, especially in the anteroapical
region, but was less extensive than in fibrofatty ARVC and FaRV.
Conclusions—ARVC is a familial arrhythmogenic disease
characterized by fibrofatty replacement of myocytes with scattered foci
of inflammation. Fat infiltration per se is probably a different
process that should not be considered synonymous with ARVC.
© 1998 American Heart Association, Inc.
Clinical Investigation and Reports
Arrhythmogenic Right Ventricular Cardiomyopathy and Fatty Replacement of the Right Ventricular Myocardium
Are They Different Diseases?
Key Words: arrhythmia • death, sudden • cardiomyopathy
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