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肥厚型梗阻性心肌病的病理特征。

Pathological features of hypertrophic obstructive cardiomyopathy.

作者信息

Davies M J, Pomerance A, Teare R D

出版信息

J Clin Pathol. 1974 Jul;27(7):529-35. doi: 10.1136/jcp.27.7.529.

Abstract

The macroscopic features of hypertrophic obstructive cardiomyopathy are variable. The most easily recognized picture is of disproportionate and asymmetrical left ventricular hypertrophy with a small ventricular volume. Symmetrical ventricular hypertrophy also occurs and dilatation of the ventricular cavity may lead to a configuration more usually associated with congestive cardiomyopathy. Papillary muscle involvement leads to a bullet shape, often retained even when the ventricle dilates. Eighteen of the hearts showed a distinctive band of fibrous thickening below the aortic valve. This was a mirror image of the free edge of the anterior mitral cusp, had the microscopic features of an endocardial friction lesion, and was clearly the morphological expression of the systolic contact between cusp and septum seen on cineangiography. This band is characteristic of hypertrophic obstructive cardiomyopathy; it was more common in older patients and is of particular diagnostic value in cases with symmetrical hypertrophy, including those with dilated ventricular cavities. Sudden death was the commonest presentation in the younger cases but in several cases over 60 years at death hypertrophic obstructive cardiomyopathy was an incidental necropsy finding.

摘要

肥厚型梗阻性心肌病的宏观特征各不相同。最容易识别的表现是左心室肥厚不成比例且不对称,心室容积较小。也会出现对称性心室肥厚,心室腔扩张可能导致一种更常见于充血性心肌病的形态。乳头肌受累会导致心脏呈子弹状,即使心室扩张时通常也会保留这种形状。18颗心脏在主动脉瓣下方显示出一条独特的纤维增厚带。这是二尖瓣前叶游离缘的镜像,具有心内膜摩擦病变的微观特征,显然是血管造影术中看到的瓣叶与室间隔之间收缩期接触的形态学表现。这条带是肥厚型梗阻性心肌病所特有的;在老年患者中更常见,在对称性肥厚的病例中具有特殊的诊断价值,包括心室腔扩张的病例。猝死是年轻病例最常见的表现,但在一些60岁以上死亡的病例中,肥厚型梗阻性心肌病是尸检时偶然发现的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a66d/475391/134b58c88803/jclinpath00129-0015-a.jpg

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