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无肥厚型肥厚型心肌病:两个心肌排列紊乱但心肌质量未增加的家系。

Hypertrophic cardiomyopathy without hypertrophy: two families with myocardial disarray in the absence of increased myocardial mass.

作者信息

McKenna W J, Stewart J T, Nihoyannopoulos P, McGinty F, Davies M J

机构信息

Department of Cardiological Sciences, St George's Hospital Medical School, London.

出版信息

Br Heart J. 1990 May;63(5):287-90. doi: 10.1136/hrt.63.5.287.

DOI:10.1136/hrt.63.5.287
PMID:2278798
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1024478/
Abstract

Two families are described in which individuals showed widespread myocardial disarray at histological examination, in the absence of macroscopic cardiac hypertrophy. In one family the clinical presentation was that of sudden unexpected cardiac death in four family members; members of the other family presented with electrocardiographic repolarisation changes and abnormalities of left ventricular diastolic function. The finding of myocardial disarray, the characteristic histological abnormality of hypertrophic cardiomyopathy, in the absence of increased cardiac mass suggests a wider range of abnormality in hypertrophic cardiomyopathy than is currently recognised.

摘要

本文描述了两个家族,其中个体在组织学检查中显示广泛的心肌排列紊乱,但无明显的心脏肥大。在一个家族中,四名家族成员临床表现为意外猝死;另一个家族的成员则表现为心电图复极改变和左心室舒张功能异常。在无心脏质量增加的情况下发现心肌排列紊乱,即肥厚型心肌病的特征性组织学异常,提示肥厚型心肌病的异常范围比目前公认的更广。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0706/1024478/404cb55970d7/brheartj00053-0027-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0706/1024478/1bb1faa97b6e/brheartj00053-0026-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0706/1024478/404cb55970d7/brheartj00053-0027-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0706/1024478/1bb1faa97b6e/brheartj00053-0026-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0706/1024478/404cb55970d7/brheartj00053-0027-a.jpg

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本文引用的文献

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HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY.肥厚型梗阻性心肌病
Br Heart J. 1964 Jan;26(1):16-32. doi: 10.1136/hrt.26.1.16.
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Myocardial disarray and fibrosis across hypertrophic cardiomyopathy stages associate with ECG markers of arrhythmic risk.
肥厚型心肌病各阶段的心肌排列紊乱和纤维化与心律失常风险的心电图标志物相关。
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Clinical dilemmas in predicting the progression of pre-clinical hypertrophic cardiomyopathy-is MRI strain the solution?预测临床前期肥厚型心肌病进展中的临床困境——磁共振成像应变是解决办法吗?
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Myoarchitectural disarray of hypertrophic cardiomyopathy begins pre-birth.肥厚型心肌病的肌构筑紊乱始于出生前。
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Identification of a Multiplex Biomarker Panel for Hypertrophic Cardiomyopathy Using Quantitative Proteomics and Machine Learning.使用定量蛋白质组学和机器学习鉴定肥厚型心肌病的多重生物标志物面板。
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Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. A wide angle, two dimensional echocardiographic study of 125 patients.肥厚型心肌病左心室肥厚的分布模式及意义。对125例患者的广角二维超声心动图研究。
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