Morimoto Shin-ichiro, Sekiguchi Morie, Uemura Akihisa, Hiramitsu Shinya, Kimura Katsutomo, Ohtsuki Masatsugu, Ishii Junichi, Kato Shigeru, Kasanuki Hiroshi, Hishida Hitoshi
Division of Cardiology, Department of Internal Medicine, Fujita Health University School of Medicine, Toyoake, Aichi, Japan.
Jpn Heart J. 2003 Jul;44(4):505-13. doi: 10.1536/jhj.44.505.
Apical hypertrophic cardiomyopathy has been divided into two entities: apical asymmetric septal hypertrophy (apical ASH) and apical symmetric hypertrophy (AH). The latter differs clinically from hypertrophic cardiomyopathy (HCM) with ASH, and it is unclear whether AH represents a distinct subtype of HCM. In the present study, the presence or absence and the extent of cardiac muscle cell disorganization, a histologic characteristic of HCM, were compared in patients with AH (n = 10) and ASH (n = 29) in whom cardiac biopsy specimens were obtained from the left ventricular apex and interventricular septum. Disorganization was graded as (1+) in only 1 patient in the AH group and (-) in the remaining 9. In contrast, in the ASH group disorganization was graded as (1+) in 15 patients, (2+) in 7, (3+) in 3, and (-) in only 4 (P < 0.0001). Thus, it was observed that in AH disorganization is virtually absent or at most limited to a very narrow area. It is concluded from a histological stand point as well that the type of apical hypertrophic cardiomyopathy showing apical symmetric hypertrophy differs from usual HCM.
心尖不对称性室间隔肥厚(心尖部ASH)和心尖对称性肥厚(AH)。后者在临床上与伴有心尖部ASH的肥厚型心肌病(HCM)不同,目前尚不清楚AH是否代表HCM的一种独特亚型。在本研究中,对10例AH患者和29例ASH患者进行了比较,这些患者均接受了左心室心尖和室间隔心肌活检,以观察HCM的组织学特征——心肌细胞排列紊乱的有无及程度。AH组仅1例患者的排列紊乱程度为(1+),其余9例为(-)。相比之下,ASH组15例患者的排列紊乱程度为(1+),7例为(2+),3例为(3+),仅4例为(-)(P<0.0001)。因此,可以观察到AH几乎不存在排列紊乱,或最多仅限于非常狭窄的区域。从组织学角度也可以得出结论,表现为心尖对称性肥厚的心尖肥厚型心肌病与普通HCM不同。