PREVALENCE AND CHARACTERISTICS OF DISPROPORTIONATE VENTRICULAR SEPTAL THICKENING IN PATIENTS WITH ACQUIRED OR CONGENITAL HEART-DISEASES - ECHOCARDIOGRAPHIC AND MORPHOLOGIC FINDINGS
PREVALENCE AND CHARACTERISTICS OF DISPROPORTIONATE VENTRICULAR SEPTAL THICKENING IN PATIENTS WITH ACQUIRED OR CONGENITAL HEART-DISEASES - ECHOCARDIOGRAPHIC AND MORPHOLOGIC FINDINGS
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DOI：
10.1161/01.cir.55.3.489
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发表时间：
1977-01-01
期刊：
影响因子：
37.8
通讯作者：
EPSTEIN, SE
中科院分区：
文献类型：
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作者：
MARON, BJ;CLARK, CE;EPSTEIN, SE
对304例各种心脏病患者进行了超声心动图和尸检。不成比例的室间隔增厚(间隔与游离壁比率)的总体流行率。1.3)为10%。与心脏病变类型有关。肺动脉狭窄或原发性肺动脉高压的患病率较高(20%)，艾森门格综合征或主动脉瓣或二尖瓣病变的患病率较低(15%)，而房间隔或室间隔缺损者则不存在。右室负荷过重时，室间隔不成比例增厚的发生率与室壁收缩压升高有关。在尸检研究的16例不成比例室间隔增厚的患者中，没有一例显示出明显的室间隔心肌细胞定向障碍，其特征是遗传传递的非对称性室间隔肥厚(ASH)。在59例室间隔不成比例增厚和相关心脏病患者的一级亲属中，超声心动图只有1例显示出不成比例的室间隔增厚。与其他心脏疾病相关的不成比例的室间隔增厚通常是由继发性肥厚引起的，并不是遗传性ASH的表现。
Echocardiographic and necropsy studies were performed in 304 patients with various cardiac diseases. The overall prevalence of disproportionated ventricular septal thickening (septal to free wall ratio .gtoreq. 1.3) was 10%. It was related to the type of cardiac lesion. Prevalence was high (> 20%) in pulmonary stenosis or primary pulmonary hypertension, lower (< 15%) in Eisenmenger syndrome or aortic or mitral valvular disease and was not present in atrial or ventricular septal defect. In right ventricular overload, prevalence of disproportionate septal thickening correlated with increasing ventricular systolic pressure. None of 16 patients with disproportionate septal thickening studied at necropsy showed marked disorientation of cardiac muscle cells in the ventricular septum, characteristic of genetically transmitted asymmetric septal hypertrophy (ASH). Disproportionate septal thickening was demonstrated by echocardiography in only 1 of 59 first degree relatives of patients with disproportionate septal thickening and associated cardiac diseases. Disproportionate ventricular septal thickening associated with other cardiac diseases usually was due to secondary hypertrophy and was not a manifestation of genetically transmitted ASH.