心肌病 心肌炎課件

心血管系統(tǒng)疾病動脈粥樣硬化、冠心病高血壓風(fēng)濕病感染性心內(nèi)膜炎心瓣膜病心肌病和心肌炎心血管系統(tǒng)疾病MedicalCollegeofXiamenUniversity2CARDIOMYOPATHY心肌病Cardiomyopathyreferstoprimarydiseaseofthemyocardiumand,inthissense,excludesmyocardialdiseasecausedbyischemia,hypertension,valvulardysfunction,congenitalanomalies,orinflammatorydisorders.Heart-musclediseaseofunknowncause.PrimaryCardiomyopathy,IdiopathicCardiomyopathyMedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity3

Cardiomyopathyisdividedintothreeforms:DilatedCardiomyopathy(DMC)(90%)HypertrophicCardiomyopathy(HMC)RestrictiveCardiomyopathy(RMC)MedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity4Thetermdilatedcardiomyopathy(DCM)isappliedtoaformofcardiomyopathycharacterizedbyprogressivecardiacdilationandcontractile(systolic)dysfunction,usuallywithconcomitanthypertrophy.Itissometimescalledcongestivecardiomyopathy.DilatedCardiomyopathy(90%)MedicalCollegeofXiamenUniv心肌病和心肌炎心肌病(cardiomyopathy)病因不明的以心肌病變?yōu)橹鞯囊活愋呐K病。

DilatedCardiomyopathyHypertrophicCardiomyopathyRestrictiveCardiomyopathy心肌病和心肌炎心肌病(cardiomyopathy)擴張性心肌病正常心臟體積增大、重量增加、心尖鈍圓擴張性心肌病正常心臟體積增大、重量增加、心尖鈍圓兩側(cè)心腔明顯擴張，心室壁略厚兩側(cè)心腔明顯擴張，心室壁略厚心肌肥大心肌肥大心肌肥大心肌肥大心肌細胞肥大，心肌間質(zhì)纖維化臨床心力衰竭猝死

心肌細胞肥大，臨床MedicalCollegeofXiamenUniversity11ClinicalFeatures

DCMmayoccuratanyage,includinginchildhood,butitmostcommonlyaffectsindividualsbetweentheagesof20and50.

ItpresentswithslowlyprogressivesignsandsymptomsofCHF慢性心衰

suchasshortnessofbreath,easyfatigability,andpoorexertionalcapacity.

Deathisusuallyattributabletoprogressivecardiacfailureorarrhythmiaandcanoccursuddenly.

Cardiactransplantationisfrequentlydone.MedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity12HypertrophicCardiomyopathy(HCM)Hypertrophiccardiomyopathy(HCM)ischaracterizedbymyocardialhypertrophy,poorlycompliantleftventricularmyocardium（左室順應(yīng)性差）

leadingtoabnormaldiastolicfilling,andinaboutonethirdofcases,intermittentventricularoutflowobstruction（流出道梗阻）.MedicalCollegeofXiamenUniv肥厚性心肌病室間隔顯著增厚、乳頭肌肥厚、心腔狹窄,重量增加肥厚性心肌病室間隔顯著增厚、乳頭肌肥厚、MedicalCollegeofXiamenUniversity14MedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity15(A)Theinterventricularseptumisthickerthanthefreewalloftheleftventricleandimpingesoftheoutflowtract.(B)Asectionofthemyocardiumshowsmyofiberdisarray（心肌纖維走行紊亂）characterizedbyanobliqueandoftenperpendicularorientationofadjacenthypertrophicmyocytes.MedicalCollegeofXiamenUniv肥厚性心肌病心肌肥大排列紊亂間質(zhì)纖維化肥厚性心肌病MedicalCollegeofXiamenUniversity17ThemajorclinicalproblemsinHCMareatrialfibrillation房顫,muralthrombusformationleadingtoembolizationandpossiblestroke,intractable頑固性

cardiacfailure,ventriculararrhythmias,and,notinfrequently,suddendeath,especiallyinsomeaffectedfamilies.Indeed,HCMisoneofthemostcommoncausesofsudden,otherwiseunexplaineddeathinyoungathletes.MedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity18

患者女性，39歲，以“勞累后胸悶氣促近1年”入院。緣于1年前無明顯誘因出現(xiàn)勞累后胸悶、氣促，休息后可緩解，尚可爬3層樓，近1年來有5~6次暈厥史。心臟超聲提示“室間隔明顯增厚，左室流出道狹窄，二尖瓣前葉SAM征陽性，左房增大，主動脈瓣及二尖瓣輕度返流，左室順應(yīng)性減退”。入院診斷“肥厚型心肌?。汗Ｗ栊汀?/p>

行“二尖瓣置換術(shù)＋左室流出道疏通術(shù)”，于右無冠瓣下切除肥厚之室間隔，約1×0.8cm。

MedicalCollegeofXiamenUnivFigure3.SchematicDiagramofaPatientUndergoingSurgicalSeptalMyectomy.Beforetheoperation,thereisseverehypertrophyofthebasalseptum,withsystolicanteriormotionofthemitralvalve(PanelA).Thisresultsinsevereoutflowtractobstructionaswellasmitralregurgitation.Duringsurgery(PanelB),theportionofthebasalseptumthatprojectsintotheoutflowtractisremovedbyascalpel,resultinginabolitionoftheoutflowtractobstruction(PanelC).Inaddition,thereisnolongersystolicanteriormotionofthemitralvalve,andthemitralregurgitationisabolished.Figure3.SchematicDiagramofMedicalCollegeofXiamenUniversity20RestrictiveCardiomyopathy(RCM)Restrictivecardiomyopathyisadisordercharacterizedbyaprimarydecreaseinventricularcompliance,resultinginimpairedventricularfillingduringdiastole.MedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity21Impairmentofcompliance(diastolicdysfunction)

Impairmentofcompliance(diastolicdysfunction)

Impairmentofcontractility(systolicdysfunction)

MedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity22Sarcomere肌(原纖維)節(jié),肌小節(jié)Asymmetrical不對稱的

MedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity23MedicalCollegeofXiamenUniv克山病心肌嚴重變性、壞死和瘢痕形成。克山病心肌嚴重變性、壞死和瘢痕形成。克山病心室壁變薄，心室壁散在的瘢痕灶，肉柱間附壁血栓?？松讲⌒氖冶谧儽。氖冶谏⒃诘鸟：墼?，MedicalCollegeofXiamenUniversity26MyocarditisMyocarditisisageneralizedinflammationofthemyocardiumassociatedwithnecrosisanddegenerationofmyocytes.Numerousinfectiousagentscancausemyocarditis,butthemajorityarebelievedtobeviral,suchasCoxsackieBvirus.ThepathogenesisofviralmyocarditisisthoughttoinvolvedirectviralcytotoxicityorTcell-mediatedimmunereactionsdirectedagainstinfectedmyocytes.MedicalCollegeofXiamenUnivMedicalCollegeofXiamenUniversity27ViralmyocarditisThemyocardialfibersaredisruptedbyaprominentinterstitialinfiltrateoflymphocytesandmacrophages.Myocarditiscanoccuratanyage,anditisoneofthefewheartdiseasethatcanproduceacuteheartfailureinpreviouslyhealt