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孤立性左心室心肌致密化不全:心肌发育异常的病例

Isolated left ventricular non-compaction: the case for abnormal myocardial development.

作者信息

Breckenridge Ross A, Anderson Robert H, Elliott Perry M

机构信息

Department of Clinical Pharmacology, BHF Laboratories, University College, London.

出版信息

Cardiol Young. 2007 Apr;17(2):124-9. doi: 10.1017/S1047951107000273. Epub 2007 Feb 26.

DOI:10.1017/S1047951107000273
PMID:17319979
Abstract

Isolated ventricular non-compaction is an increasingly commonly diagnosed myocardial disorder characterised by excessive and prominent trabeculation of the morphologically left, and occasionally the right, ventricle. This is associated with high rates of thromboembolism, cardiac failure, and cardiac arrhythmia. Recent improvements in understanding the embryonic processes underlying ventricular formation have led to the hypothesis that ventricular non-compaction is due to a failure of normal ventriculogenesis, leading to abnormal myocardium which may present clinically many years later. Experimental work in animal models provides several candidate transcription factors and signalling molecules that could, in theory, cause ventricular non-compaction if disrupted.

摘要

孤立性心室肌致密化不全是一种越来越常见的被诊断出的心肌疾病,其特征是形态学上的左心室,偶尔也包括右心室,出现过度且明显的肌小梁。这与高血栓栓塞率、心力衰竭和心律失常相关。最近在理解心室形成的胚胎过程方面取得的进展引发了这样一种假说,即心室肌致密化不全是由于正常心室发生过程失败,导致异常心肌,而这种异常心肌可能在多年后才出现临床症状。动物模型的实验研究提供了几种候选转录因子和信号分子,理论上,如果这些因子和分子受到干扰,可能会导致心室肌致密化不全。

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