[Multiple myeloma manifesting as cardiac insufficiency].
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Two new noninvasive imaging modalities, electron beam computed tomography (EBCT) and magnetic resonance imaging(MRI), have emerged in recent years and have provided images of the heart with high temporal and spatial resolution. They provides accurate information of myocardial thickness, ventricular shape and volume, wall motion, and ventricular function in patients with cardiomyopathy. In addition, myocardial damage can be evaluated using contrast media. They can give unique diagnostic information above and beyond that of more traditional noninvasive modalities such as two-dimensional echocardiography, left ventriculography and radionuclide technique because both modalities acquire images in three dimensions. They have a possibility to become an alternative modality of the left ventriculography in the diagnosis of cardiomyopathy.
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These heart muscle diseases, associated with systolic or diastolic dysfunction, are associated with a high prevalence and morbidity, because of the frequent evolution towards congestive heart failure or sudden death. Dilated cardiomyopathy is the first cause of heart transplantation and Hypertrophic cardiomyopathy is the first cause of sudden death in athletes. Familial and monogenic forms of these diseases are frequent and this should lead to a systematic cardiological familial inquest in first degree relatives of a patient. Although they were previously called as "idiopathic", the pathophysiology of the disease is actually under investigation thanks to molecular genetics. This completely new knowledge should lead to the development of new therapeutic strategies.
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Myofibrillar myopathy (MFM) is a rare autosomal dominant disorder characterized by cardiac and skeletal myopathy. Either of these can dominate in the clinical picture. It is associated with cardiomyopathy, arrhythmia and/or atrioventricular (AV) conduction defects. Myofibrillar myopathy is often an overlooked disorder because of its variable clinical presentation. We highlight the various cardiovascular manifestations of MFM that have been reported in the literature and address the importance of considering this syndrome in young patients presenting with idiopathic cardiomyopathy and/or AV conduction defects.
Thymic cysts are rare and almost always asymptomatic. The authors report the case of a 45 year old woman with a thymic cyst diagnosed after recurrent right sided heart failure resulting in signs suggestive of adiastole, regressing after "pleural" (mainly cystic) aspiration and diuretic therapy without any morphological or functional changes on Doppler echocardiography. This report concerns a rare tumour, with an exceptional volume (2 litres) extending down the cardiac borders and causing cardiac compression. It illustrates the diagnostic difficulty of a pathology with an unusual clinical presentation, despite complementary investigations including CT scan and MRI, very sensitive in this type of problem. A complete cure was obtained by total surgical ablation.