Related Experiment Videos
[Endocardial fibroelastosis in children to one year of age]
M Sobaniec-Zotowska1, S Sulkowski, W Sobaniec
1Zakładu Anatomii Patologicznej AM, Białymstoku.
Insights
Endocardial fibroelastosis (EFE) affected 10.6% of congenital heart disease cases in infants. This study highlights EFE
Area of Science:
- Pediatric Pathology
- Cardiovascular Diseases
- Congenital Abnormalities
Context:
- Endocardial fibroelastosis (EFE) is a rare congenital heart disease.
- Autopsy data from 2398 newborns and infants (1976-1985) were analyzed.
- EFE represented 10.6% of congenital heart diseases in this cohort.
Purpose:
- To investigate the incidence, characteristics, and potential etiopathogenesis of endocardial fibroelastosis in infants.
- To analyze morphological features and associated conditions of EFE.
- To assess the clinical diagnostic accuracy of EFE.
Summary:
- Thirty-two cases of EFE were identified, most frequent in infants aged 3-6 months.
- EFE occurred in both male and female infants, with 62.5% being primary and 37.5% secondary.
- Associated conditions included joint, bone, and respiratory abnormalities. Etiopathogenesis may involve congenital factors, myocardial infection, or collagenic disease. The disease course was severe, with low clinical diagnostic rates (18.75%).
Impact:
- Provides crucial data on EFE prevalence and associations in an infant autopsy cohort.
- Suggests potential etiological pathways, including congenital origin, infection, and collagenic disease.
- Underscores the diagnostic challenges and severity of EFE in infants.
Abstract:
Out of 2398 autopsies of newborns and infants performed between 1976 and 1985, thirty two cases of endocardial fibroelastosis were found, i.e. 10.6% of the congenital heart diseases in this age group. Endocardial fibroelastosis was most frequent (44%) in infants aged between 3 and 6 months. Seventeen cases of the disease were seen in male infants and 15 cases in female infants. Twenty cases of the endocardial fibroelastosis (62.5%) were of isolated character (primary) whereas 12 cases (37.5%) were of the secondary character associated with other heart abnormalities. Nine cases (about 28%) coexisted with congenital abnormalities of joint and bone system and respiratory tract. A morphologic analysis of the endocardial fibroelastosis with particular reference to its etiopathogenesis suggest its congenital origin or (and) an effect of infection on myocardium. It was also suggested that endocardial fibroelastosis may be a symptom of collagenic disease. About 70% of autopsied infants were hospitalized over 3 days what meant that the course of the disease was severe. Endocardial fibroelastosis was diagnosed clinically only in 6 cases (18.75%).