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  Česky / Czech version Čs. Pediat., 54, 1999, No. 8, p. 417-420.
 
Fate of Children with Endocardial Fibroelastosis in Adult Age 
Augustínová A. 1 , Benedeková M. 2 , Mašura J. 1 

 


Summary:

       39 children from a group of 62 patients who suffered as infants from cardiac decompensation and had on ECG left ventricular decompensation with an overload reached adult age. The diagnosis of FE was assessed by analogy with 18 infants with a similar clinical picture who died and necropsy revealed FE. 70% of the surviving children have a normal clinical finding and good quality of life. In half of them there are however liminal findings of left ventricular dysfunction on echocardiography, which in the authors’ opinion suggests persistance of pathological structures in the endocardium with possible relapse of decompensation under certain adverse conditions of the organism. The 30% who survive to adult age have some other pathological clinical findings, most frequently persisting mitral insufficiency. The authors consider the latter patients as not completely cured. Four patients who died in adult age, one after transplantation of the heart and two with serious relapse of decompensation after childbirth draw attention to the risks of patients with FE. It is questionable whether there is a possibility of complete cure and elimination of the pathological structure of the endocardium.

        Key words: fibroelastosis of the endocardium (FE), intravital diagnosis, survival to adult age
       

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