RT-207
|
Congestive heart failure: a not
so rare manifestation of arrythmogenic right ventricular cardiomyopathy
|
|
|
Cristina Basso, Annalisa
Angelini, Gaetano Thiene, Ugolino Livi, Andrea Nava, Sergio Dalla Volta.
Department of Pathology, Cardiovascular Surgery and Cardiology, University of
Padua Medical School, Padua, Italy
|
|
Abstract
|
|
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
is a heart muscle disease of unknown etiology which is characterized by myocardial atrophy
with fatty or fibrofatty replacement. In some instances pathologic changes are so
widespread to affect the whole RV, with or without left ventricular involvement, as to
determine congestive heart failure (CHF). In our anatomical collection of ARVC heart
specimens, 25% belonged to patients with CHF, with a mean age of 42 years, significantly
higher than that of the remaining cases (24 years, p < 001). An in vivo ARVC diagnosis
was achieved in 6, whereas the remaining 4 were considered affected by dilated
cardiomyopathy. Four had previous episodes of systemic thromboembolism. Four were autopsy
observations whereas the remaining six underwent cardiac transplantation because of
refractory CHF. Pathologic study revealed an increased heart weight, with RV enlargement
in all; RV aneurysms in 80%; mural thrombosis of the RV in 2 and of left atrial appendage
in 1. A left ventricular involvement was present in 90% with apical aneurysm in 2. At
histology all cases showed fibrofatty ARVC variety with patchy inflammatory infiltrates.
In conclusion, CHF may be the end-point in the natural history of ARVC. Age-dependancy
of this clinical outcome supports the theory of an acquired progressive disease. In the
setting of CHF due to biventricular depressed contractility, ARVC may mimic dilated
cardiomyopathy and heart transplantation constitutes the only therapeutic option.
|
Key Words
|
|
Arrhythmogenic right ventricular dysplasia
congestive heart failure, dilated cardiomyopathy, wall motion abnormalities, rest,
exercise, heart transplantation, OA
|
