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Paediatric dilated cardiomyopathy with and without endocardial fibroelastosis - a pathological analysis of 89
Jeanette A Reyes1, Anne I Dipchand2, David A Chiasson1
1Department of Paediatric Laboratory Medicine, Hospital for Sick Children, Toronto, Canada.
Insights
Endocardial fibroelastosis is common in pediatric dilated cardiomyopathy, especially in infants. This finding may warrant a new designation for further research into its significance.
Area of Science:
- Pediatric Cardiology
- Cardiac Pathology
- Cardiovascular Surgery
Background:
- Dilated cardiomyopathy is a primary reason for pediatric heart transplants.
- Endocardial fibroelastosis (EFE) is a recognized pathological finding in pediatric dilated cardiomyopathy, but its prognostic importance is unclear.
Purpose of the Study:
- To investigate the association between left ventricular endocardial fibroelastosis (LV-EFE) and pediatric dilated cardiomyopathy.
- To characterize pathological findings in dilated cardiomyopathy explants across different age groups.
- To compare the frequency of LV-EFE in pediatric dilated cardiomyopathy patients.
Main Methods:
- Retrospective review of surgical pathology reports for dilated cardiomyopathy explants from 1986 to 2016.
- Analysis of 89 explants (47 males, 42 females) characterized by increased weight and left ventricular dilatation.
- Comparison of LV-EFE prevalence across four age groups: <1 year, 1-5 years, 6-10 years, and >11 years.
Main Results:
- All explants showed increased weight and left ventricular dilatation without increased wall thickness.
- Left ventricular endocardial fibroelastosis was present in 95% of specimens in the two youngest age groups (<1 year and 1-5 years).
- Infants (<1 year) had a 19-fold increased odds, and children aged 1-5 years had a 6-fold increased odds of LV-EFE compared to the oldest group (>11 years).
Conclusions:
- Endocardial fibroelastosis is a highly prevalent pathological finding in pediatric dilated cardiomyopathy, particularly in infants and young children.
- Explant hearts with and without LV-EFE were otherwise similar in phenotype.
- A new clinico-pathological designation, "Dilated Cardiomyopathy with Endocardial Fibroelastosis," is proposed to encourage further research into LV-EFE's prognostic and therapeutic implications.
Abstract:
Heart failure due to dilated cardiomyopathy is a major indication for paediatric cardiac transplantation. Endocardial fibroelastosis is a recognised pathological finding of unknown prognostic significance in paediatric dilated cardiomyopathy. To evaluate the nature of the association between left ventricular endocardial fibroelastosis and paediatric dilated cardiomyopathy, we reviewed surgical pathology reports of dilated cardiomyopathy explants (1986-2016) in order to characterise the pathological findings and to compare and contrast their frequency among four age groups: less than 1 year; 1-5 years; 6-10 years; and greater than 11 years. The 89 explants (47 males and 42 females) were all characterised by increased weight and left ventricular chamber dilatation without increased wall thickness. Ninety-five per cent of the specimens in the two youngest subsets had left ventricular endocardial fibroelastosis. Compared to the oldest age group, recipients aged 1-5 years had a 6-fold increase and those younger than 1 year a 19-fold increase in the odds of observing left ventricular endocardial fibroelastosis. Explants with and without endocardial fibroelastosis were otherwise phenotypically similar. In paediatric dilated cardiomyopathy endocardial fibroelastosis is a very common pathological finding, especially in infants and young children. We propose that the descriptive, clinico-pathological designation "Dilated Cardiomyopathy with Endocardial Fibroelastosis" should be adopted to facilitate future investigation into the potential prognostic/therapeutic significance of left ventricular endocardial fibroelastosis.
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