[Endocardial fibroelastosis (E.F.) and its differential diagnosis].
The endocardial fibroelastosis (EFE) is the most frequent cardiomyopathy. This disease is characterised by endocardial hyperplasia due to proliferation of elastic and collagenous fibres. There are primary and secondary forms. Within the primary form, the infantile form is the most frequent and of greatest importance to the pediatrician. This form is more a syndrom than a distinct disease. It is a reaction of the endocard due to several noxes. Lately a possible viral etiology is being discussed e.g. Parotitis, Coxsackie or other viruses. Clinical criteria for diagnosis are: cardiomegaly, left ventricular hypertrophy seen in 97% in the ECG, the absence of a murmur (or a soft apical mumur) absence of cyanosis and absence of systemic disease. Differential diagnosis is mainly between fibroplastic parietal endocarditis (FPE), cardiovascular collagenosis (CC) and endomyocard fibrosis (EMF). In FPE thrombosis is frequent and typically there is eosinophilia. CC is found in South Africa and is characterised by edema and fibrinoid necrosis. MEF is present mainly in Uganda, Nigeria and South India, characterised by endocardial fibrosis, valve involvement and eosinophilia. The obstructive hypertrophic cardiomyopathy is characterised by a pronounced cardiomegaly, insufficient weight gain as well as dyspnea and cyanosis. Catheterization shows a gradient across one or both of the outflow tracts due to hypertrophic subaortic or subpulmonic stenosis. Therapy of EFE consists in treating the cardiac decompensation and according to the severity of the disease, in steroids.
D009202:Cardiomyopathies / Q000175:diagnosis; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis; D002318:Cardiovascular Diseases / Q000175:diagnosis; D002404:Catheterization; D002648:Child; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000175:diagnosis; D004695:Endocardial Fibroelastosis / Q000175:diagnosis* / Q000188:drug therapy; D004696:Endocarditis / Q000175:diagnosis; D004719:Endomyocardial Fibrosis / Q000175:diagnosis; D004770:Enterovirus; D004802:Eosinophilia / Q000175:diagnosis; D006801:Humans; D007223:Infant; D011241:Prednisone / Q000627:therapeutic use; D011657:Pulmonary Eosinophilia / Q000175:diagnosis; D013256:Steroids / Q000627:therapeutic use; D013577:Syndrome; D013927:Thrombosis / Q000175:diagnosis
D013256:Steroids; D011241:Prednisone
Die Fibroelastosis Endocardica (F.E.) und ihre Differentialdiagnose