[The cardiomyopathy of idiopathic hemochromatosis].
A retrospective study of the case histories of 216 patients with idiopathic haemochromatosis has highlighted the frequency of cardiac involvement in this condition (53%). Two forms can be distinguished: a latent one (65%), in which the changes are predominantly electrocardiographic, and a clinical form (35%) with the features of congestive cardiomyopathy, notable for the rapidity of onset after right heart failure, the degree of cardiomegaly, the constant finding of abnormalities of ventricular repolarisation, the relative frequency of latent disorders of supra-His atrio-ventricular conduction, and the finding of elongation of the isovolumic contraction time on the phonomechanocardiogram. A haemodynamic profile is the same as for non-obstructive hypotonic cardiomyopathies, and is usually associated with a slow rise in left ventricular pressure. The cardiomyopathy, which is the most frequent cause of death, determines the prognosis in this condition. It may be found in association with diabetes and gonad failure. The finding of cardiomyopathy indicates basic treatment by veresection, which may be the only means of establishing a favourable outcome.
D000328:Adult; D000368:Aged; D009202:Cardiomyopathies / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology; D004562:Electrocardiography; D006329:Heart Conduction System / Q000503:physiopathology; D006432:Hemochromatosis / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology; D006439:Hemodynamics; D006801:Humans; D008875:Middle Aged; D009200:Myocardial Contraction; D010701:Phonocardiography; D011674:Pulse
La myocardiopathie de l'hémochromatose idiopathique.