Koga Y, Takahashi H, Ifuku M, Itaya M, Adachi K, Toshima H
J Cardiogr. 1984 Aug;14(2):301-10.
Clinical and morphologic features are described in a subgroup of 22 patients with hypertrophic cardiomyopathy, who showed ventricular septal hypertrophy localized to the apical region (apical asymmetric septal hypertrophy: apical ASH). All patients had ventricular septal thickness of 17 mm or less with an average of 13 +/- 3 mm in the M-mode echocardiograms. In contrast, the two-dimensional echocardiograms demonstrated septal hypertrophy localized to the apical region, with an average septal thickness of 20 +/- 3 mm at the mitral valve and papillary muscle levels. On the left ventriculogram, 82% of patients with apical ASH showed inward concavity of the right-inferior wall of the left ventricle, indicating hypertrophy of the ventricular septum. However, no patient showed spade-like appearance of the left ventricle and only two showed giant T wave inversion exceeding 10 mm. Echocardiographic examination performed in eight affected relatives revealed typical asymmetric septal hypertrophy (ASH) in four relatives, one of them showing a resting pressure gradient of 30 mmHg in the left ventricle. The remaining four relatives showed similar apical ASH. When compared with patients of typical ASH, patients with apical ASH demonstrated significantly greater cardiac size, left ventricular end-diastolic pressure, left atrial and left ventricular diameters and significantly lower percent fractional shortening. Atrial fibrillation, B bump of the mitral echogram and heart failure were more frequent in this subgroup of patients. Thus, apical ASH appeared to be a part of the morphologic spectrum of hypertrophic cardiomyopathy with ASH and to be a separate disease entity from apical hypertrophy previously described in Japan. Severely impaired diastolic performances associated with mild to moderately depressed systolic function of the left ventricle were the characteristic clinical features of the subgroup of patients with apical ASH.
对22例肥厚型心肌病患者的亚组进行了临床和形态学特征描述，这些患者表现为局限于心尖区域的室间隔肥厚（心尖不对称性室间隔肥厚：心尖ASH）。所有患者在M型超声心动图中室间隔厚度均为17mm或更小，平均为13±3mm。相比之下，二维超声心动图显示室间隔肥厚局限于心尖区域，在二尖瓣和乳头肌水平平均室间隔厚度为20±3mm。在左心室造影中，82%的心尖ASH患者左心室右后壁向内凹陷，提示室间隔肥厚。然而，没有患者表现出左心室铲状外观，只有2例表现出超过10mm的巨大T波倒置。对8名受累亲属进行的超声心动图检查显示，4名亲属有典型的不对称性室间隔肥厚（ASH），其中1名在静息状态下左心室压力阶差为30mmHg。其余4名亲属表现出类似的心尖ASH。与典型ASH患者相比，心尖ASH患者的心脏大小、左心室舒张末期压力、左心房和左心室直径显著更大，而缩短分数百分比显著更低。该亚组患者房颤、二尖瓣超声心动图B波和心力衰竭更为常见。因此，心尖ASH似乎是肥厚型心肌病伴ASH形态学谱的一部分，并且是一种与日本先前描述的心尖肥厚不同的疾病实体。左心室舒张功能严重受损伴轻度至中度收缩功能降低是心尖ASH患者亚组的特征性临床特点。
