心肌病 心肌炎课件.ppt

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1、心血管系统疾病动脉粥样硬化、冠心病高血压风湿病感染性心内膜炎 心瓣膜病心肌病和心肌炎,Medical College of Xiamen University,2,CARDIOMYOPATHY心肌病,Cardiomyopathy refers to primary disease of the myocardium and,in this sense,excludes myocardial disease caused by ischemia,hypertension,valvular dysfunction,congenital anomalies,or inflammatory disor

2、ders.Heart-muscle disease of unknown cause.Primary Cardiomyopathy,Idiopathic Cardiomyopathy,Medical College of Xiamen University,3,Cardiomyopathy is divided into three forms:Dilated Cardiomyopathy(DMC)(90%)Hypertrophic Cardiomyopathy(HMC)Restrictive Cardiomyopathy(RMC),Medical College of Xiamen Univ

3、ersity,4,The term dilated cardiomyopathy(DCM)is applied to a form of cardiomyopathy characterized by progressive cardiac dilation and contractile(systolic)dysfunction,usually with concomitant hypertrophy.It is sometimes called congestive cardiomyopathy.,Dilated Cardiomyopathy(90%),心肌病和心肌炎,心肌病(cardio

4、myopathy)病因不明的以心肌病变为主的一类心脏病。Dilated Cardiomyopathy Hypertrophic Cardiomyopathy Restrictive Cardiomyopathy,扩张性心肌病,正常心脏,体积增大、重量增加、心尖钝圆,两侧心腔明显扩张，心室壁略厚,心 肌 肥 大,心 肌 肥 大,心肌细胞肥大，心肌间质纤维化,临床 心力衰竭 猝死,Medical College of Xiamen University,11,Clinical Features DCM may occur at any age,including in childhood,but

5、it most commonly affects individuals between the ages of 20 and 50.It presents with slowly progressive signs and symptoms of CHF慢性心衰 such as shortness of breath,easy fatigability,and poor exertional capacity.Death is usually attributable to progressive cardiac failure or arrhythmia and can occur sud

6、denly.Cardiac transplantation is frequently done.,Medical College of Xiamen University,12,Hypertrophic Cardiomyopathy(HCM),Hypertrophic cardiomyopathy(HCM)is characterized by myocardial hypertrophy,poorly compliant left ventricular myocardium（左室顺应性差）leading to abnormal diastolic filling,and in about

7、 one third of cases,intermittent ventricular outflow obstruction（流出道梗阻）.,肥厚性心肌病,室间隔显著增厚、乳头肌肥厚、心腔狭窄,重量增加,Medical College of Xiamen University,14,Medical College of Xiamen University,15,(A)The interventricular septum is thicker than the free wall of the left ventricle and impinges of the outflow tract

8、.(B)A section of the myocardium shows myofiber disarray（心肌纤维走行紊乱）characterized by an oblique and often perpendicular orientation of adjacent hypertrophic myocytes.,肥厚性心肌病心肌肥大排列紊乱间质纤维化,Medical College of Xiamen University,17,The major clinical problems in HCM are atrial fibrillation房颤,mural thrombus

9、formation leading to embolization and possible stroke,intractable顽固性 cardiac failure,ventricular arrhythmias,and,not infrequently,sudden death,especially in some affected families.Indeed,HCM is one of the most common causes of sudden,otherwise unexplained death in young athletes.,Medical College of

10、Xiamen University,18,患者女性，39岁，以“劳累后胸闷气促近1年”入院。缘于1年前无明显诱因出现劳累后胸闷、气促，休息后可缓解，尚可爬3层楼，近1年来有56次晕厥史。心脏超声提示“室间隔明显增厚，左室流出道狭窄，二尖瓣前叶SAM征阳性，左房增大，主动脉瓣及二尖瓣轻度返流，左室顺应性减退”。入院诊断“肥厚型心肌病：梗阻型”行“二尖瓣置换术左室流出道疏通术”，于右无冠瓣下切除肥厚之室间隔，约10.8cm。,Figure 3.Schematic Diagram of a Patient Undergoing Surgical Septal Myectomy.Before the

11、operation,there is severe hypertrophy of the basal septum,with systolic anterior motion of the mitral valve(Panel A).This results in severe outflow tract obstruction as well as mitral regurgitation.During surgery(Panel B),the portion of the basal septum that projects into the outflow tract is remove

12、d by a scalpel,resulting in abolition of the outflow tract obstruction(Panel C).In addition,there is no longer systolic anterior motion of the mitral valve,and the mitral regurgitation is abolished.,Medical College of Xiamen University,20,Restrictive Cardiomyopathy(RCM),Restrictive cardiomyopathy is

13、 a disorder characterized by a primary decrease in ventricular compliance,resulting in impaired ventricular filling during diastole.,Medical College of Xiamen University,21,Impairment of compliance(diastolic dysfunction),Impairment of compliance(diastolic dysfunction),Impairment of contractility(sys

14、tolic dysfunction),Medical College of Xiamen University,22,Sarcomere肌(原纤维)节,肌小节Asymmetrical不对称的,Medical College of Xiamen University,23,克山病,心肌严重变性、坏死和瘢痕形成。,克山病,心室壁变薄，心室壁散在的瘢痕灶，肉柱间附壁血栓。,Medical College of Xiamen University,26,Myocarditis,Myocarditis is a generalized inflammation of the myocardium ass

15、ociated with necrosis and degeneration of myocytes.Numerous infectious agents can cause myocarditis,but the majority are believed to be viral,such as Coxsackie B virus.The pathogenesis of viral myocarditis is thought to involve direct viral cytotoxicity or T cell-mediated immune reactions directed a

16、gainst infected myocytes.,Medical College of Xiamen University,27,Viral myocarditisThe myocardial fibers are disrupted by a prominent interstitial infiltrate of lymphocytes and macrophages.Myocarditis can occur at any age,and it is one of the few heart disease that can produce acute heart failure in

17、 previously healthy adolescents.,Medical College of Xiamen University,28,化脓性心包炎,Medical College of Xiamen University,29,Fibrinous pericarditis.The heart of a patient who died in uremia displays a shaggy,fibrinous exudate covering the visceral pericardium.,Constrictive pericarditis.The heart is encased in a fibrotic thickened,and adherent pericardium.,Medical College of Xiamen University,30,Cardiac myxoma.The left atrium contains a large,polypoid tumor that protrudes into the mitral valve orifice.,