Fate of Children with Endocardial Fibroelastosis in Adult Age
Osud detí s fibroelastózou endokardu v dospelosti
Zo súboru 62 pacientov, ktorí boli ako dojčatá kardiálne dekompenzovaní a mali na EKG hypertrofiu ľavejkomory s preťažením, 39 detí prežilo do dospelosti. Diagnóza FE bola stanovená na analógii s 18 z nich, ktorís rovnakým klinickým obrazom exitovali a na pitve sa dokázala FE. 70 % žijúcich má normálny klinický náleza dobrú kvalitu života. U polovice z nich sú však hraničné nálezy dysfunkcie ľavej komory na ECHOKG, čo autoripokladajú za pretrvávanie patologických štruktúr v endokarde s možnosťou recidívy dekompenzácie za určitej,nepriaznivej situácie organizmu. 30 % pacientov, ktorí prežívajú do dospelosti, má niektoré ďalšie patologickéklinické nálezy, najčastejšie ide o pretrvávajúcu mitrálnu insuficienciu. Týchto autori považujú za neúplnevyliečených. Štyria pacienti, ktorí exitovali v dospelosti, 1 po transplantácii srdca a 2 so závažnou recidívoudekompenzácie po pôrode, upozorňujú na riziká v živote pacientov s FE. Otázkou ostáva možnosť úplnéhovyliečenia, resp. vymiznutia patologickej štruktúry endokardu.
Klíčová slova:
fibroelastóza endokardu (FE), intravitálna diagnostika, prežívanie do dospelosti
Authors:
A. Augustínová 1; M. Benedeková 2; J. Mašura 1
Authors‘ workplace:
Detské kardiocentrum DFNsP, Bratislava, 1prednosta doc. MUDr. J. Mašura, CSc. I. detská klinika DFNsP, Bratislava, 2prednostka doc. MUDr. M. Benedeková, CSc.
Published in:
Čes-slov Pediat 1999; (8): 417-420.
Category:
Overview
39 children from a group of 62 patients who suffered as infants from cardiac decompensation and had on ECGleft ventricular decompensation with an overload reached adult age. The diagnosis of FE was assessed by analogywith 18 infants with a similar clinical picture who died and necropsy revealed FE. 70% of the surviving childrenhave a normal clinical finding and good quality of life. In half of them there are however liminal findings of leftventricular dysfunction on echocardiography, which in the authors’ opinion suggests persistance of pathologicalstructures in the endocardium with possible relapse of decompensation under certain adverse conditions of theorganism. The 30% who survive to adult age have some other pathological clinical findings, most frequentlypersisting mitral insufficiency. The authors consider the latter patients as not completely cured. Four patients whodied in adult age, one after transplantation of the heart and two with serious relapse of decompensation afterchildbirth draw attention to the risks of patients with FE. It is questionable whether there is a possibility of completecure and elimination of the pathological structure of the endocardium.
Key words:
fibroelastosis of the endocardium (FE), intravital diagnosis, survival to adult age
Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
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