[Fabry's disease. Biochemical and clinical study of an Alsacian family].
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Biomedical subjects
Publications and source records attributed to J M Mossard.
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The total ejection fraction of the left ventricle is studied in 37 patients, most of them with coronary disease, according to two methods: contrast angiography in a single plane and angioscintigraphy (first passage and balanced), in order to compare the results according to the presence or the absence of dyskinesias. The two methods are performed in succession according to the usual views. The population is divided in two sub-groups according to the presence (17 patients) or the absence (20 patients) of segmental abnormalities. Segmental abnormalities are divided into 11 antero-apical aneurysms and 6 infero-posterior aneurysms. The correlations of the ejection fractions calculated according to the two methods and the two views, are usually satisfactory in the two sub-groups and especially as the parietal kinetics is homogeneous. In case of segmental abnormalities of the contraction, there are variations in the total ejection fractions depending on the technique and the views that are used, but the differences are not significant.
We are reporting the case of a young man presenting repeated syncopes linked to episodes of ventricular tachycardia. The rhythm disorders were due to the existence of a papyraceous right ventricle with concomitant involvement of the left ventricle disclosed during the etiological work-up. We are reporting, at this time, the usual diagnostic elements of this disease, but mostly original data provided by the nuclear magnetic resonance, as the clinical examination and the EKG are often less revealing except for late potentials always found in the arrhythmic form. The right, ventricular dysplasia and the papyraceous right ventricle represent two very close entities. Particular characteristics enable to differentiate them and also the differential diagnosis with other diseases are discussed by stressing the advantage of magnetic resonance imaging, a new method, non invasive, which lends itself well to the analysis of cardiac morphology.
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