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左侧右心室心肌病

Left side right ventricular cardiomyopathy.

作者信息

Michalodimitrakis M, Papadomanolakis A, Stiakakis J, Kanaki K

机构信息

Department of Forensic Sciences, University of Crete, Heraklion, Greece.

出版信息

Med Sci Law. 2002 Oct;42(4):313-7. doi: 10.1177/002580240204200406.

DOI:10.1177/002580240204200406
PMID:12487516
Abstract

Arrhythmogenic right ventricular cardiomyopathy or dysplasia, a heart muscle disease of unknown cause, is anatomically characterized by variable replacement of myocardial muscle with adipose or fibroadipose tissue. It is usually considered a selective disorder whereas concomitant left ventricular involvement has been noted in a few cases. Two cases of the disease with evidence of extensive left ventricular involvement at pathologic examination are described. Hearts from two patients who died suddenly showed extensive biventricular infiltration by fibrofatty tissue in the first case and exclusively in the wall of the left ventricle the localization of the fatty and fibrotic lesions. These findings might suggest that the various localizations of the fibroadipose tissue are rather different expressions of the same disease and it is preferable to be termed 'arrhythmogenic cardiomyopathy' as other studies also indicate.

摘要

致心律失常性右心室心肌病或发育异常是一种病因不明的心肌疾病,其解剖学特征是心肌组织被脂肪或纤维脂肪组织不同程度地替代。它通常被认为是一种选择性疾病,不过少数病例中也发现有左心室受累。本文描述了两例经病理检查证实有广泛左心室受累的该疾病病例。第一例中,两名猝死患者的心脏显示双心室均有广泛的纤维脂肪组织浸润;第二例中,脂肪和纤维化病变仅局限于左心室壁。这些发现可能表明,纤维脂肪组织的不同定位是同一疾病的不同表现形式,正如其他研究也表明的那样,最好将其称为“致心律失常性心肌病”。

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引用本文的文献

1
State of the Art Review on Genetics and Precision Medicine in Arrhythmogenic Cardiomyopathy.心律失常性心肌病的遗传学和精准医学的最新研究进展综述。
Int J Mol Sci. 2020 Sep 10;21(18):6615. doi: 10.3390/ijms21186615.
2
Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes.致心律失常性心肌病:电学和结构表型
Arrhythm Electrophysiol Rev. 2016 Aug;5(2):90-101. doi: 10.15420/AER.2016.4.3.
3
Arrhythmogenic ventricular cardiomyopathy: A paradigm shift from right to biventricular disease.致心律失常性右室心肌病:从右心室疾病到双心室疾病的范式转变。
World J Cardiol. 2014 Apr 26;6(4):154-74. doi: 10.4330/wjc.v6.i4.154.
4
Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy.组织驻留Sca1+ PDGFRα+间充质祖细胞是致心律失常性心肌病中纤维脂肪浸润的细胞来源。
F1000Res. 2013 Jun 19;2:141. doi: 10.12688/f1000research.2-141.v1. eCollection 2013.
5
Pathophysiology of arrhythmogenic cardiomyopathy.心律失常性心肌病的病理生理学。
Nat Rev Cardiol. 2011 Nov 29;9(4):223-33. doi: 10.1038/nrcardio.2011.173.
6
Sudden adult death.成人猝死。
Forensic Sci Med Pathol. 2009;5(3):210-32. doi: 10.1007/s12024-009-9099-3. Epub 2009 Jul 18.