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Paediatric dilated cardiomyopathy with and without endocardial fibroelastosis – a pathological analysis of 89 explants
Cardiology in the Young ( IF 1 ) Pub Date : 2021-09-06 , DOI: 10.1017/s1047951121003590
Jeanette A Reyes 1 , Anne I Dipchand 2 , David A Chiasson 1
Affiliation  

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.



中文翻译:

伴有和不伴有心内膜弹性纤维增生的小儿扩张型心肌病——89 种外植体的病理学分析

扩张型心肌病引起的心力衰竭是小儿心脏移植的主要适应症。心内膜弹性纤维增生是公认的病理发现,在小儿扩张型心肌病中具有未知的预后意义。为了评估左心室心内膜弹性纤维增生症和小儿扩张型心肌病之间关联的性质,我们回顾了扩张型心肌病外植体(1986-2016 年)的外科病理学报告,以描述病理学发现并比较和对比它们在四个年龄组中的频率:少于1年;1-5年;6-10年;并且大于 11 年。89 个外植体(47 名男性和 42 名女性)均以重量增加和左心室扩张而不增加壁厚为特征。在两个最年轻的亚组中,95% 的样本患有左心室心内膜弹性纤维增生症。与最年长的年龄组相比,1-5 岁的受者观察到左心室心内膜弹性纤维增生的几率增加了 6 倍,而小于 1 岁的受者观察到的几率增加了 19 倍。具有和不具有心内膜弹性纤维增生的外植体在其他方面具有相似的表型。在小儿扩张型心肌病中,心内膜弹性纤维增生是一种非常常见的病理表现,尤其是在婴儿和幼儿中。我们建议应采用描述性、临床病理学名称“伴有心内膜弹力纤维增生的扩张型心肌病”,以促进未来对左心室心内膜弹力纤维增生症潜在预后/治疗意义的研究。

更新日期:2021-09-06
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