《内科学》课程PPT教学课件（循环系统）第十章 心肌病 Cardiomyopathy

心肌病↓Cardiomyopathy王丽石河子大学医学院一附院心内二科

Cardiomyopathy 心 肌 病 王 丽 石河子大学医学院一附院心内二 科

Definition“"A primary disorder of the heart musclethat causes abnormal myocardial performanceand is not the result of disease or dysfunctionof other cardiac structures ... myocardialinfarction, systemic hypertension, valvularstenosis or regurgitation

Definition “A primary disorder of the heart muscle that causes abnormal myocardial performance and is not the result of disease or dysfunction of other cardiac structures . myocardial infarction, systemic hypertension, valvular stenosis or regurgitation

心肌病特点总结心病因不明*心肌功能障碍*与特异性心肌病(原因已知)鉴别

心肌病特点总结 ❖病因不明 ❖心肌功能障碍 ❖与特异性心肌病(原因已知)鉴别

WHOClassification(1995年根据病理生理学DCM)1）扩张型心肌病（Dilated，2)肥厚型心肌病（HypertrophicHCM)3)RCM).限制型心肌病(Restrictive,4）致心律失常型右室心肌病（ARVD/C另有不定型的心肌病

WHO Classification (1995年根据病理生理学) 1）扩张型心肌病(Dilated , DCM). 2) 肥厚型心肌病(Hypertrophic HCM). 3）限制型心肌病(Restrictive, RCM). 4）致心律失常型右室心肌病(ARVD/C) 另有不定型的心肌病

特异性心肌病---Specific heart muscledisease(secondary)>感染性：细菌，病毒，支原体，真菌等>代谢性：甲亢，甲低,糖尿病，脚气病>全身性:SLE，内分湿性关节炎，硬皮病>遗传性，家族性：假性肥大型肌萎缩>过敏性及中毒性：酒精，磺胺，阿霉素

特异性心肌病-Specific heart muscle disease (secondary) ➢感染性:细菌,病毒,支原体,真菌等 ➢代谢性 :甲亢,甲低,糖尿病,脚气病 ➢全身性 :SLE,内分湿性关节炎,硬皮病 ➢遗传性，家族性:假性肥大型肌萎缩 ➢过敏性及中毒性:酒精,磺胺,阿霉素

扩张型心肌病Idiopathic Dilated CardiomyopathyIDC

扩张型心肌病 Idiopathic Dilated Cardiomyopathy IDC

IDC - DefinitionJ al disease of unknown etiology that principally affectsthe myocardium LV dilatation and systolic dysfunctionpathologyincreased heart size and weightventricular dilatation, normal wall thicknessheart dysfunction out of portion to fibrosis

IDC - Definition ▪ a disease of unknown etiology that principally affects the myocardium ▪ LV dilatation and systolic dysfunction ▪ pathology ➢ increased heart size and weight ➢ ventricular dilatation, normal wall thickness ➢ heart dysfunction out of portion to fibrosis

病因1病毒感染；2）基因及自身免疫（发现本病与组织相容性有关）；3）遗传，代谢，中毒等

病因 1）病毒感染； 2）基因及自身免疫（发现本病与组织相容性有 关）； 3）遗传，代谢，中毒等

Incidence and Prognosis3-10 cases per 100,00020,000 new cases per year in the U.S.A.death from progressive pump failure25%1-year35-40%2-year40-80%（现存活率可达70%）5-yearstabilization observed in 20-50% of patientcomplete recovery is rare

Incidence and Prognosis ◼ 3-10 cases per 100,000 ◼ 20,000 new cases per year in the U.S.A. ◼ death from progressive pump failure 1-year 25% 2-year 35-40% 5-year 40-80%（现存活率可达７０％） ◼ stabilization observed in 20-50% of patient ◼ complete recovery is rare

Idiopathic Dilated CardiomyopathyDbserved Survival of 104 Patients1201041008072605651+454037+35+312420+19·160570123468910YearsAmJCardiol1981:47:525

Idiopathic Dilated Cardiomyopathy Observed Survival of 104 Patients 104 7 2 5 6 5 1 4 5 3 7 3 5 3 1 2 4 1 9 1 6 0 2 0 4 0 6 0 8 0 100 120 0 1 2 3 4 5 6 7 8 9 1 0 Years Am J Cardiol 1981; 47:525