MORPHOLOGIC FEATURES OF HYPERTROPHIC CARDIOMYOPATHY

肥厚型心肌病的形态学特征

• 批准号: 3899227
• 负责人: W C ROBERTS
• 金额: --
• 依托单位: NATIONAL HEART, LUNG, AND BLOOD INSTITUTE
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/3899227
• 关键词: heart disorder diagnosis; human subject; hypertrophic myocardiopathy; morphology; pathology; postmortem

The morphologic features of 220 patients with hypertrophic cardiomyopathy (HC) were described. The major gross morphologic features of HC, in order of decreasing frequency, are as follows: 1 ) dilated atria (100%); 2) increased heart weight (95%); 3) nondilated left ventricle (79%); 4) thickened mitral valve (75%); 5) fibrous endocardial mural plaque, left ventricular outflow tract (72%); and 6) a ventricular septum thicker than the left ventricular free wall (60%). Gross left ventricular fibrosis occurred in 84% of the patients and rarely was a consequence of associated narrowing of an epicardial coronary artery disease. HC may cause death in any decade of life. The diagnosis of HC in the very young (age <10 years) and in the very old (age >70) is more difficult clinically and at necropsy than in the intermediate age groups. Patients with the obstructive type of HC usually have larger hearts, a higher frequency of a fibrous plaque in the left ventricular outflow tract, and a higher frequency of a thickened mitral valve than those without obstruction. Left ventricular cavity dilation is present in about 20% of patients with HC and is a result of either operation (with survival >43 months) or extensive left ventricular wall scarring.

220例肥厚型心肌病的形态学特征 (HC)被描述。HC的主要大体形态学特征， 发生率依次为：1）心房扩张（100%）; 心脏重量增加（95%）; 3）左心室未扩张（79%）; 4） 二尖瓣增厚（75%）; 5）纤维性内膜斑块，左侧 心室流出道（72%）;和6）室间隔厚于 左室游离壁（60%）。左心室纤维化 发生在84%的患者中，很少是相关的结果。 心外膜冠状动脉狭窄HC可能导致死亡， 任何十年的生活。HC的诊断在非常年轻的（年龄<10岁） 在非常老的（年龄> 70）是更困难的临床和尸检 在中间年龄组中。梗阻型肺动脉高压患者 HC通常有较大的心脏， 左心室流出道，以及更高频率的增厚 二尖瓣口狭窄者较无狭窄者明显。左室腔 扩张存在于约20%的HC患者中，并且是 手术（生存期> 43个月）或广泛左心室 墙上的疤痕