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Hypertrophic Cardiomyopathy

心血管

关键词心血管 疾病 遗传性心肌病

词汇介绍

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解析

Hypertrophic /,haipə'trɔfik/

释    义   adj. 肥厚的;过度膨胀的

同根词   hypertrophied adj.过度增大的 hypertonicity n. 高张性;过度紧张;高渗性

例    句   Objective To explore the relationship between exercise hypertension and heart damage or hypertrophic left ventricle.

目的:研究运动高血压与心肌缺血、左室肥厚的相关性。

 

cardiomyopathy 英/,kɑːdɪəʊmaɪ'ɒpəθɪ/    美 /,kɑrdɪomaɪ'ɑpəθi/


释    义   n. a disorder (usually of unknown origin) of the heart muscle (myocardium)(尤指原发性的)心肌症,心肌病

词    源   cardio myopathy

例  句 Objective: To study the related factors of peripartum cardiomyopathy(PPCM) patients outcomes for echocardiography characteristics.

目的:探寻影响围生期心肌病患者临床转归的超声因素。

概述

肥厚型心肌病是由心脏肌节蛋白基因突变引起的一种遗传性心肌病,左室壁肥厚程度、部位和分布呈异质性表型,临床过程也呈多样性,包括猝死、心衰和脑卒中。成人普通人群HCM患病率为1/500。 组织学结果肌细胞结构紊乱,包括肌细胞肥大、排列紊乱、细胞核畸形,壁内冠状动脉异常,以及间质性和替代性纤维化 临床表现尽管很多肥厚型心肌病患者没有症状,但是患者也会出现下列一种或多种症状:劳力性呼吸困难、端坐呼吸、阵发性夜间呼吸困难、胸痛、心悸、晕厥先兆/晕厥、体位性头晕、乏力或水肿。体格检查

Clinical Course and Management of Hypertrophic Cardiomyopathy复制标题

肥厚型心肌病的临床病程及治疗

发表时间:2018-11-15

影响因子:79.3

作者: Barry J. Maron

期刊:N. Engl. J. Med

Hypertrophic cardiomyopathy (HCM) is a genetic cardiomyopathy caused by mutations of the cardiac sarcomere, resulting in heterogeneous phenotypic expression with respect to the extent, location, and distribution of left ventricle (LV) wall thickening as well as a diverse clinical course including sudden death, heart failure, and stroke. The prevalence of HCM in the general population is 1 in 500 adults. Histopathology in patients with HCM reveals disorganized myocyte architecture, including hypertrophied myocytes in a disarray pattern with bizarre-shaped nuclei, abnormal intramural coronary arteries, and interstitial as well as replacement fibrosis.While many patients with HCM remain asymptomatic, it is not uncommon for patients to develop one or more of the following symptoms: dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea, chest pain, palpitations, presyncope/syncope, postural lightheadedness, fatigue, or edema. The evaluation for HCM in an individual patient may be prompted by a family history of HCM, systolic ejection murmur, abnormal 12-lead electrocardiogram showing otherwise unexplained evidence of LV hypertrophy (LVH), and clinical symptoms including syncope. In addition to performing a comprehensive cardiac history and physical examination and an electrocardiogram (ECG) in all patients with suspected HCM, cardiac imaging to identify LVH should be performed. In the patient presenting with LVH, HCM must be distinguished from acquired causes of cardiac hypertrophy, including hypertension, aortic stenosis, and athlete's heart. Other than the dynamic subaortic (LVOT) obstruction seen in HCM, several other anatomic and physiologic abnormalities can be associated with increased pressure gradients between the LV and the aorta, including volume depletion, subaortic stenosis, and valvular aortic stenosis.

译文

肥厚型心肌病(HCM)是由心肌肌节突变引起的遗传性心肌病,导致左心室(LV)壁增厚的程度,位置和分布以及包括猝死在内的多种临床过程中的异质表型表达,心力衰竭和中风。一般人群中HCM的患病率为500名成人中的1名。 HCM患者的组织病理学显示紊乱的肌细胞结构,包括处于混乱模式的肥大肌细胞,具有奇异形状的核,异常的壁内冠状动脉,间质以及替代纤维化。虽然许多HCM患者仍无症状,但患者并不少见。出现以下一种或多种症状:运动时呼吸困难,端坐呼吸,阵发性夜间呼吸困难,胸痛,心悸,晕厥前期/晕厥,姿势性头晕,疲劳或水肿。 HCM的家族史,收缩期射血杂音,异常的12导联心电图显示LV肥大(LVH)的其他无法解释的证据,以及包括晕厥在内的临床症状,可能会对个体患者的HCM进行评估。除了对所有疑似HCM患者进行全面的心脏病史和体格检查以及心电图(ECG)外,还应进行心脏成像以识别LVH。 在患有LVH的患者中,必须将HCM与获得性心脏肥大的原因(包括高血压,主动脉瓣狭窄和运动员的心脏)区分开来。除HCM中观察到的动态主动脉下(LVOT)阻塞外,其他一些解剖学和生理学异常可能与LV和主动脉之间的压力梯度增加有关,包括体积减少,主动脉瓣下狭窄和瓣膜主动脉瓣狭窄。