INTRAMURAL CORONARY ARTERY DISEASE IN HYPERTROPHIC CARDIOMYOPATHY

肥厚型心肌病中的壁内冠状动脉疾病

• 批准号: 3966737
• 负责人: B J MARON
• 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/3966737
• 关键词: coronary disorder; echocardiography; human tissue; hypertrophic myocardiopathy; pathology

Many patients with hypertrophic cardiomyopathy have signs and symptoms of myocardial ischemia and dysfunction. To determine the prevalence and extent as well as the clinical relevance of abnormal intramural coronary arteries, a histologic analysis of left ventricular myocardium obtained at necropsy was performed in 48 patients with hypertrophic cardiomyopathy and in 68 controls. In hypertrophic cardiomyopathy, abnormal intramural coronary arteries were characterized by thickening of the vessel wall and a decrease in luminal size. The wall thickening was due to proliferation of medial and/or intimal components, particularly smooth muscle cells and collagen. Of the 48 patients with hypertrophic cardiomyopathy, 40 (83%) had abnormalities of intramural coronary arteries located in the ventricular septum (33 patients), anterior left ventricular free wall (20 patients) or posterior free wall (9 patients); an average of 3.0 plus/minus 0.7 abnormal arteries were identified per tissue section. Altered intramural coronary arteries were also significantly more common in tissue sections having considerable myocardial fibrosis (31 of 42, 74%) than in those with no or mild fibrosis (31 of 102, 30%; p less than 0.001). In contrast, only rare altered intramural coronary arteries were identified in 6 (9%) of the 68 control patients, and those arteries showed only mild thickening of the wall and minimal luminal narrowing (abnormal arteries per section, 0.1 plus/minus 0.05; p less than 0.001). Hence, abnormal intramural coronary arteries with markedly thickened walls and narrowed lumens are present in increased numbers in most patients with hypertrophic cardiomyopathy at necropsy and they may represent a congenital component of the underlying cardiomyopathic process.

许多肥厚型心肌病患者都有 心肌缺血和功能障碍。以确定流行率和 室壁内冠状动脉异常的范围及临床意义 动脉，左室心肌的组织学分析 对48例肥厚型心肌病患者进行了尸检。 在68名对照组中。肥厚型心肌病室壁异常 冠状动脉的特征是管壁增厚和 管腔尺寸减小。壁厚是由于血管内皮细胞增殖所致。 中膜和/或内膜成分，特别是平滑肌细胞和 胶原蛋白。在48例肥厚型心肌病患者中，40例(83%) 有壁内冠状动脉异常位于 室间隔33例，左前壁游离壁20例 患者)或后游离壁(9名患者)；平均为3.0加/减 每层组织切片共检出异常动脉0.7条。更改后的 壁间冠状动脉在组织中也明显更常见。 有明显心肌纤维化的切片(31/42，74%)比 无纤维化组或轻度纤维化组(31/102，30%；P<0.001)。在……里面 相比之下，只有罕见的室壁内冠状动脉改变被发现。 68例对照组患者中有6例(9%)，且这些动脉仅显示为轻度 管壁增厚和微小的管腔狭窄(每支异常动脉 节，0.1正负0.05%；p小于0.001)。因此，不正常 壁内冠状动脉壁明显增厚和狭窄 大多数肥厚性疾病患者管腔数目增多。 尸检时的心肌病，可能是先天性心脏病的一个组成部分 潜在的心肌病过程。