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Paediatric dilated cardiomyopathy with and without endocardial fibroelastosis – a pathological analysis of 89 explants

Published online by Cambridge University Press:  06 September 2021

Jeanette A. Reyes Open the ORCID record for Jeanette A. Reyes [Opens in a new window] ,
Anne I. Dipchand  and
David A. Chiasson
Jeanette A. Reyes*
Affiliation:
Department of Paediatric Laboratory Medicine, Hospital for Sick Children, Toronto, Canada
Anne I. Dipchand
Affiliation:
Heart Centre – Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Canada
David A. Chiasson
Affiliation:
Department of Paediatric Laboratory Medicine, Hospital for Sick Children, Toronto, Canada
*
Author for correspondence: J. A. Reyes, Department of Paediatric Laboratory Medicine, Division of Pathology, Hospital for Sick Children, 555 University, Avenue, Room 3133A – Elm Wing, Toronto, Ontario, Canada. Tel: 416-813-5973. E-mail: j.reyes@sickkids.ca
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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.

Keywords

Dilated cardiomyopathyendocardial fibroelastosisheart failurecardiac transplantationpaediatric
Type
Original Article
Information
Cardiology in the Young , Volume 32 , Issue 7 , July 2022 , pp. 1041 - 1047
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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