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A CLINICOPATHOLOGICAL STUDY OF 100 CASES OF RIGHT VENTRICULAR...
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发布时间：2021-03-25
A CLINICOPATHOLOGICAL STUDY OF 100 CASES OF RIGHT VENTRICULAR HYPOPLASIA (INCLUDING 89 CASES OF SUDDEN UNEXPECTED DEATH) - RELATED TO UHLS ANOMALY Author: TABIB, ALOIRE, R
Journal: ARCHIVES DES MALADIES DU COEUR ET DES VAISSEAUX
Issue Date: 1992
Abstract(summary):
The authors present the pathologist\'s view of Uhl\'s anomaly based on 100 cases recorded over 31 years, 89 of which came from medico-legal autopsies following sudden unexpected death in young subjects. The anatomic diagnosis is made by macroscopic examination of the whole heart in diffuse forms (parchment heart) and from transverse sections of the mid third of the ventricle in incomplete forms, showing absence of myocardium in the juxtaseptal anterior wall of the right ventricle. The wall entirely made up of adipose tissue, may retain its normal thickness. On microscropy, normal adipose tissue is observed between the epicardum and endocardium, sometimes with islets of myocardium dispersed in the subendocardial layer and with, in about half the cases, subendocardial bundles of non-hyalinised collagen without any inflammatory cellular infiltration. Other structural histopathological lesions may complicate the malformation, involving the intraseptal arterioles or the conductive system. In addition to the 89 cases of sudden and unexpected death before the age of 50 (preceded by some modification of the patient\'s life style in 29 cases), 11 cases were symptomatic and 5 were transplanted with a good result. The clinical diagnosis of Uhl\'s disease is important because of its consequences : ventricular tachycardia and possible sudden death, more rarely cardiac failure with age. Histological analysis should take into account the constant presence of adipose tissue in the right ventricle, and should only interprete excessive quantities with associated myocytic aplasia as pathological. The congenital nature of Uhl\'s anomaly would seem to be established after recent genetic studies : this feature is one of the differences with >, an acquired disease interpreted as a localis-ed parietal lesion following myocarditis, ischemia, etc. The partial forms of Uhl\'s anomaly are a difficult anatomical entity to diagnose clinically from > sudden death of young subjects and which are opposed to sudden deaths of patients with cardiac failure or severe coronary artery disease.
Page: 1789---1795
Journal: ARCHIVES DES MALADIES DU COEUR ET DES VAISSEAUX
Issue Date: 1992
Abstract(summary):
The authors present the pathologist\'s view of Uhl\'s anomaly based on 100 cases recorded over 31 years, 89 of which came from medico-legal autopsies following sudden unexpected death in young subjects. The anatomic diagnosis is made by macroscopic examination of the whole heart in diffuse forms (parchment heart) and from transverse sections of the mid third of the ventricle in incomplete forms, showing absence of myocardium in the juxtaseptal anterior wall of the right ventricle. The wall entirely made up of adipose tissue, may retain its normal thickness. On microscropy, normal adipose tissue is observed between the epicardum and endocardium, sometimes with islets of myocardium dispersed in the subendocardial layer and with, in about half the cases, subendocardial bundles of non-hyalinised collagen without any inflammatory cellular infiltration. Other structural histopathological lesions may complicate the malformation, involving the intraseptal arterioles or the conductive system. In addition to the 89 cases of sudden and unexpected death before the age of 50 (preceded by some modification of the patient\'s life style in 29 cases), 11 cases were symptomatic and 5 were transplanted with a good result. The clinical diagnosis of Uhl\'s disease is important because of its consequences : ventricular tachycardia and possible sudden death, more rarely cardiac failure with age. Histological analysis should take into account the constant presence of adipose tissue in the right ventricle, and should only interprete excessive quantities with associated myocytic aplasia as pathological. The congenital nature of Uhl\'s anomaly would seem to be established after recent genetic studies : this feature is one of the differences with >, an acquired disease interpreted as a localis-ed parietal lesion following myocarditis, ischemia, etc. The partial forms of Uhl\'s anomaly are a difficult anatomical entity to diagnose clinically from > sudden death of young subjects and which are opposed to sudden deaths of patients with cardiac failure or severe coronary artery disease.
Page: 1789---1795
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发布于 ： 2021-03-25
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