英文教學(xué)講解課件

HEART.THEHEARTNormalPathologyHeartFailure:L,RHeartDisease Congenital:LRshunts,RLshunts,ObstrustiveIschemic:Angina,Infarction,ChronicIschemia,SuddenDeathHypertensive:Leftsided,RightsidedValvular:AS,MVP,Rheumatic,Infective,Non-Infective,Carcinoid,ArtificialValvesCardiomyopathy:Dilated,Hypertrophic,Restrictive,Myocarditis,OtherPericardium:Effusions,PericarditisTumors:Primary,EffectsofOtherPrimariesTransplants.NORMALFeatures6000L/day250-300grams40%ofalldeaths(2xcancer)?Wallthickness~pressure(i.e.,awallisonlyasthickasithastobe)?LV=1.5cmRV=0.5cmAtria=.2cmSystole/DiastoleStarling’sLaw.TERMSCARDIOMEGALYDILATATION,anychamber,orallHYPERTROPHY,andchamber,orall..STRIATIONSNUCLEUSDISCSSARCOLEMMASARC.RETIC.MITOCHONDRIAENDOTHELIUMFIBROBLASTSGLYCOGENA.N.P..S.A.NodeAVNodeBundleofHISL.Bundle,R.Bundle..AnteriorLateralPosteriorSeptal.VALVESAV:TRICUSPIDMITRALSEMILUNAR:PULMONICAORTIC.Sigmoid-shapedventricularseptumDecreasedleftventricularcavitysizeIncreasedleftatrialcavitysizeChambersBucklingofmitralleafletstowardtheleftatriumFibrousthickeningofleafletsMitralvalveannularcalcificdepositsAorticvalvecalcificdepositsValvesAtheroscleroticplaqueCalcificdepositsIncreasedcross-sectionalluminalareaTortuosityEpicardialCoronaryArteriesAmyloiddepositsBasophilicdegenerationLipofuscindepositionBrownatrophyIncreasedsubepicardialfatIncreasedmassMyocardiumCARDIACAGING.CARDIACAGINGAtheroscleroticplaqueElasticfragmentationandcollagenaccumulationSinotubularjunctioncalcificdepositsElongated(tortuous)thoracicaortaDilatedascendingaortawithrightwardshiftAorta.BROWN

ATROPHY,HEARTLIPOFUCSIN.PathologicPumpPossibilitiesPrimarymyocardialfailure(MYOPATHY)Obstructiontoflow(VALVE)Regurgitantflow(VALVE)Conductiondisorders(CONDUCTIONSYSTEM)Failuretocontainblood(WALLINTEGRITY).CHFDEFINITIONTRIAD1)TACHYCARDIA2)DYSPNEA3)EDEMAFAILUREofFrankStarlingmechanismHUMORALFACTORSCatecholamines(nor-epinephrine)?ReninAngiotensionAldosteroneAtrialNatriureticPolypeptide(ANP)?HYPERTROPHYandDILATATION.HYPERTROPHYPRESSUREOVERLOAD(CONCENTRIC)?VOLUMEOVERLOAD(CHF)LVH,RVH,atrial,etc.2Xnormalweightischemia3XnormalweightHTN>3XnormalweightMYOPATHY,aorticregurgitation...CHF:AutopsyFindingsCardiomegalyChamberDilatationHypertrophyofmyocardialfibers,BOXCARnuclei..LeftSidedFailureLowoutputvs.congestionLungspulmonarycongestionandedemaheartfailurecellsKidneyspre-renalazotemiasaltandfluidretentionrenin-aldosteroneactivationnatriureticpeptidesBrain:Irritability,decreasedattention,stuporcoma.LeftHeartFailureSymptomsDyspneaonexertionatrestOrthopnearedistributionofperipheraledemafluidgradedbynumberofpillowsneededParoxysmalNocturnalDyspnea(PND).LEFTHeartFailureDyspneaOrthopneaPND(ParoxysmalNocturnalDyspnea)?BloodtingedsputumCyanosisElevatedpulmonary“WEDGE”pressure(PCWP).RightSidedHeartFailureEtiologyleftheartfailurecorpulmonaleSymptomsandsignsLiverandspleenpassivecongestion(nutmegliver)?congestivespleenomegalyascitesKidneysPleura/PericardiumpleuralandpericardialeffusionstransudatesPeripheraltissues.RIGHTHeartFailureFATIGUE“Dependent”edemaJVDHepatomegaly(congestion)?ASCITES,PLEURALEFFUSIONGICyanosisIncreasedperipheralvenouspressure(CVP)?..HEARTDISEASECONGENITAL(CHD)?ISCHEMIC(IHD)HYPERTENSIVE(HHD)VALVULAR(VHD)MYOPATHIC(MHD).CONGENITALHEART

DEFECTSFaultyembryogenesis(week3-8)?UsuallyMONO-morphic(i.e.,SINGLElesion)(ASD,VSD,hypo-RV,hypo-LV)Maynotbeevidentuntiladultlife(Coarctation,ASD)Overallincidence1%ofUSAbirthsINCREASEDsimpleearlydetectionvianoninvasivemethods,e.g.,US,MRI,CT,etc..Tricuspidatresia1

120

Totalanomalouspulmonaryvenousconnection1

136

Truncusarteriosus4

388

Transpositionofgreatarteries4

388

Aorticstenosis4

396

Atrioventricularseptaldefect5

492

Coarctationofaorta5

577

TetralogyofFallot7

781

Patentductusarteriosus8

836

Pulmonarystenosis101043Atrialseptaldefect424482Ventricularseptaldefect%IncidenceperMillionLiveBirthsMalformation.GENETICSGeneabnormalitiesinonly10%ofCHDTrisomies21,13,15,18,XOMutationsofgeneswhichencodefortranscriptionfactorsTBX5ASD,VSD

NKX2.5ASDRegionofchromosome22importantinheartdevelopment,22q11.2deletionconotruncus,branchialarch,face.ENVIRONMENTRUBELLATERATOGENS.CHDLRSHUNTS:all“D’s”intheirnamesNOcyanosisPulmonaryhypertensionSIGNIFICANTpulmonaryhypertensionisIRREVERSIBLERLSHUNTS:all“T’s”intheirnamesCYANOSIS(i,.e.,“blue”babies)VENOUSEMBOLIbecomeSYSTEMICOBSTRUCTIONS.LRASDVSDASVDPDANONCYANOTICIRREVERSIBLEPULMONARYHYPERTENSIONISTHEMOSTFEAREDCONSEQUENCE..ASDNOTpatentforamenovaleUsuallyasymptomaticuntiladulthoodSECUNDUM(90%):DefectivefossaovalisPRIMUM(5%):NexttoAVvalves,mitralcleftSINUSVENOSUS(5%):NexttoSVCwithanomalouspulmonaryveinsdrainingtoSVCorRA

.VSDByfar,mostcommonCHDdefectOnly30%areisolatedOftenwithTETRALOGYofFALLOT90%involvethemembranousseptumIfmuscularseptumisinvolved,likelytohavemultipleholesSMALLonesoftenclosespontaneouslyLARGEonesprogresstopulmonaryhypertension..PDA90%isolatedHARSH,machinery-likemurmurLR,possiblyRLaspulmonaryhypertensionapproachessystemicpressureClosingthedefectmaybelifesavingKeepingitopenmaybelifesaving(ProstaglandinE).Why?.AVSDAssociatedwithdefective,inadequateAVvalvesCanbepartial,orCOMPLETE(ALL4CHAMBERSFREELYCOMMUNICATE)?.RLTetralogyofFallotTranspositionofgreatarteriesTruncusarteriosusTotalanomalouspulmonaryvenousconnectionTricuspidatresia.RL

SHUNTSTETRALOGYofFALLOTmostCOMMON1)VSD,large2)OBSTRUCTIONtoRVflow3)AortaOVERRIDEStheVSD4)RVHSURVIVALDEPENDSonSEVERITYofSUBPULMONICSTENOSISCanbea“PINK” tetrologyifpulmonicobstructionissmall,butthegreatertheobstruction,thegreateristheRLshunt ..TGA(TRANSPOSITION

ofGREATARTERIES)?NEEDSaSHUNTforsurvivalPDAorPFO(65%),“unstable”shuntVSD(35%),“stable”shuntRV>LVinthicknessFatalinfirstfewmonthsSurgical“switching”.TRUNCUSARTERIOSIS.TRICUSPIDATRESIAHypoplasticRVNeedsashunt,ASD,VSD,orPDAHighmortality.TotalAnomalousPulmonaryVenousConnection(TAPVC)PULMONARYVEINSdoNOTgointoLA,butintoL.innominatev.orcoronarysinusNeedsaPFOoraVSDHYPOPLASTICLA.OBSTRUCTIVECHDCOARCTATIONofaortaPulmonarystenosis/atresiaAorticstenosis/atresia.COARCTATIONofAORTAM>FButXO’sfrequentlyhaveitINFANTILEFORM(proximaltoPDA)(SERIOUS)?ADULTFORM(CLOSEDDUCTUS)?Bicuspidaorticvalve50%ofthetime.PULMONICSTENOSIS/ATRESIAIf100%atretic,hypoplasticRVwithASDClinicalseverity~stenosisseverity.AORTICSTENOSIS/ATRESIAVALVULARIfsevere,hypoplasticLVfatalSUB-valvular(subaortic)?AorticwallTHICKBELOWcuspsSUPRA-valvularAorticwallTHICKABOVEcuspsinascendingaorta.HEARTDISEASECONGENITAL(CHD)?ISCHEMIC(IHD)?HYPERTENSIVE(HHD)?VALVULAR(VHD)?MYOPATHIC(MHD)?.SYNDROMESofIHDAnginaPectoris:Stable,UnstableMyocardialInfarction(MI,AMI)?ChronicIHDCHF(CIHD)?SuddenCardiacDeath(SCD)?“Acute”CoronarySyndromes:UNSTABLEANGINAAMISCD(SuddenCardiacDeath).IHDRISKNumberofplaquesDistributionofplaquesSize,structureofplaques.ACUTECORONARYSYNDROMES“Theacutecoronarysyndromesarefrequentlyinitiatedbyanunpredictableandabruptconversionofastableatheroscleroticplaquetoanunstableandpotentiallylife-threateningatherothromboticlesionthroughsuperficialerosion,ulceration,fissuring,rupture,ordeephemorrhage,usuallywithsuperimposedthrombosis.”.EPIDEMIOLOGY?milliondieofIHDyearlyinUSA1millionin1963.Why?PreventionofcontrolcontrollableriskfactorsEarlier,betterdiagnosticmethodsPTCA,CABG,arrythmiacontrol90%ofIHDpatientshaveATHEROSCLEROSIS(nosurprisehere).ACUTECORONARYSYNDROMEFACTORS

ACUTEPLAQUECHANGE*******InflammationThrombusVasoconstriction*******MOSTIMPORTANT.ACUTEPLAQUECHANGERupture/RefissuringErosion/Ulceration,exposingECMAcuteHemorrhageNB:PlaquesdoNOThavetobeseverelystenotictocauseacutechanges,i.e.,50%ofAMIresultsfromthrombosesofplaquesshowingLESSTHAN50%stenosis..INFLAMMATIONEndothelialcellsreleaseCAMs,selectinsT-cellsreleaseTNF,IL-6,IFN-gammatostimulateandactivateendothelialcellsandmacrophagesCRPpredictstheprobabilityofdamageinanginapatients.THROMBUSTotalocclusionPartialEmbolization.VASOCONSTRICTIONCirculatingadrenergicagonistsPlateletreleaseproductsEndotheliallyreleasedfactors,suchasendothelin..Oftensmallplateletaggregatesorthrombiand/orthromboemboliFrequentUsuallysevereSuddendeathWidelyvariable,maybeabsent,partial/complete,orlysedVariableVariableSubendocardialmyocardialinfarctionOcclusiveFrequentVariableTransmuralmyocardialinfarctionNonocclusive,oftenwiththromboemboliFrequentVariableUnstableanginaNoNo>75%StableanginaPlaque-AssociatedThrombusPlaque

DisruptionStenosesSyndromeCoronaryArteryPathologyinIschemicHeartDisease.ANGINAPECTORISParoxysmal(sudden)?Recurrent15sec.15min.Reducedperfusion,butNOinfarctionTHREETYPESSTABLE:relievedbyrestornitroPRINZMETAL:SPASMismainfeature,respondstonitro,S-TelevationUNSTABLE(crescendo,PRE-infarction,Q-waveangina):perhapssomethrombosis,perhapssomenontransmuralnecrosis,perhapssomeembolization,butDISRUPTIONofPLAQUEisuniversallyagreedupon

.MYOCARDIALINFARCTIONTransmuralvs.Subendocardial(inner1/3)?DUH!EXACTSAMEriskfactorsasatherosclerosisMostareTRANSMURAL,andMOSTarecausedbycoronaryarteryocclusionInthe10%oftransmuralMIsNOTassociatedwithatherosclerosis:VasospasmEmboliUNexplained.MYOCARDIALRESPONSE>1hrMicrovascularinjury20–40minIrreversiblecellinjury40minto10%ofnormal

10minto50%ofnormal

ATPreduced<2minLossofcontractilitySecondsOnsetofATPdepletionTimeFeature.PROGRESSIONOFNECROSIS.TIMINGofGrossand

MicroscopicFindingsDensecollagenousscarScarringcomplete>2mo

Increasedcollagendeposition,withdecreasedcellularityGray-whitescar,progressivefrombordertowardcoreofinfarct2–8wk

Well-establishedgranulationtissuewithnewbloodvesselsandcollagendepositionRed-graydepressedinfarctborders10–14days

Well-developedphagocytosisofdeadcells;earlyformationoffibrovasculargranulationtissueatmarginsMaximallyyellow-tanandsoft,withdepressedred-tanmargins7–10days

Beginningdisintegrationofdeadmyofibers,withdyingneutrophils;earlyphagocytosisofdeadcellsbymacrophagesatinfarctborderHyperemicborder;centralyellow-tansoftening3–7days

Coagulationnecrosis,withlossofnucleiandstriations;interstitialinfiltrateofneutrophilsMottlingwithyellow-taninfarctcenter1–3days

Ongoingcoagulationnecrosis;pyknosisofnuclei;myocytehypereosinophilia;marginalcontractionbandnecrosis;beginningneutrophilicinfiltrateDarkmottling12–24hr

Beginningcoagulationnecrosis;edema;hemorrhageOccasionallydarkmottling4–12hrUsuallynone;variablewavinessoffibersatborderNone?–4hr.1day,3-4days,7days,weeks,months.RE-PERFUSIONThrombolysisPTCACABGReperfusionCANNOTrestorenecroticordeadfibers,onlyreversiblyinjuredonesREPERFUSION“INJURY”FreeradicalsInterleukins.AMIDIAGNOSISSYMPTOMSEKGDIAPHORESIS(10%ofMIsare“SILENT”withQ-waves)?CKMBgoldstandardenzymeTroponin-I,Troponin-TbetterCRPpredictsriskofAMIinanginapatients.COMPLICATIONSWallmotionabnormalitiesArrhythmiasRupture(4-5days)PericarditisRVinfarctionInfarctextensionMuralthrombusVentricularaneurysmPapillarymuscledysfunction(regurgitation)CHF.CIHD,aka,ischemic“cardiomyopathy”P(pán)rogresstoCHFoftenwithnopathologicorclinicalevidenceoflocalizedinfarctionExtensiveatherosclerosisNoinfarctH&Dpresent.SUDDENCARDIACDEATH350,000inUSAyearlyfromatherosclerosisNON-atheroscleroticsuddencardiacdeathincludes:CongenitalcoronaryarterydiseaseAorticstenosisMVPMyocarditisCardiomyopathy(suddendeathinyoungathletes)?PulmonaryhypertensionConductiondefectsHTN,hypertrophyofUNKNOWNetiology.AUTOPSYfindingsinSCD>75%narrowingof1-3vesselsHealedinfarcts40%“ARRHYTHMIA”isoftenaveryconvenientconclusionwhennoanatomicfindingsarepresent,i.e.,“wastebasket”diagnosis.HEARTDISEASECONGENITAL(CHD)ISCHEMIC(IHD)HYPERTENSIVE(HHD)VALVULAR(VHD)MYOPATHIC(MHD).HHD(Left)DEFINITION:Hypertrophicadaptiveresponseoftheheart,whichcanprogress:MyocardialdysfunctionCardiacdilatationCHFSuddendeath.NEEDEDforDIAGNOSIS:LVH(LV>2.0and/orHeart>500gm.)HTN(>140/90).PREVALENCE:WHAT%ofUSApeoplehavehypertension?.PREVALENCE:WHAT%ofUSApeoplehavehypertension?Answer:25%..HISTOPATHOLOGYINCREASEDFIBER(MYOCYTE)THICKNESSINCREASEDnuclearsizewithincreased“blockiness”(boxcarnucleus).CLINICALEKGSummaryofLVHCriteria

1)R-I+S-III>25mm

2)S-V1+R-V5>35mm

3)ST-Tsinleftleads

4)R-L>11mm

5)LAE+othercriteriaPositiveCriteria:1=possible2=probable3=definiteATRIAL

FIBRILLATIONWhy?*CHF,cardiacdilatation,pulmonaryvenouscongestionanddilatation..COURSE:NORMALlongevity,deathfromothercausesProgressiveIHDProgressiverenaldamage,hemorrhagicCVA(Whicharteries?)CHF.HHD(Right)=CORPULMONALEACUTE:MassivePECHRONIC:COPD,CRPD,Pulmonaryarterydisease,chestwallmotionimpairment.DiseasesofthePulmonaryParenchymaChronicobstructivepulmonarydiseaseDiffusepulmonaryinterstitialfibrosisPneumoconiosesCysticfibrosisBronchiectasisDiseasesofthePulmonaryVesselsRecurrentpulmonarythromboembolismPrimarypulmonaryhypertensionExtensivepulmonaryarteritis(e.g.,Wegenergranulomatosis)Drug-,toxin-,orradiation-inducedvascularobstructionExtensivepulmonarytumormicroembolismDisordersAffectingChestMovementKyphoscoliosisMarkedobesity(pickwickiansyndrome)NeuromusculardiseasesDisordersInducingPulmonaryArterialConstrictionMetabolicacidosisHypoxemiaChronicaltitudesicknessObstructiontomajorairwaysIdiopathicalveolarhypoventilation.HEARTDISEASECONGENITAL(CHD)ISCHEMIC(IHD)HYPERTENSIVE(HHD)VALVULAR(VHD)MYOPATHIC(MHD).ValvularHDOpeningproblems:StenosisClosingproblems:RegurgitationorIncompetence.70%ofallVHDASCalcificationofadeformedvalve“Senile”calcificASRheum,HeartDis.MSRheumaticHeartDisease.AORTICSTENOSIS 2Xgradientpressure LVH,ischemia Cardiacdecompensation,angina,CHF 50%diein5yearsifanginapresent 50%diein2yearsifCHFpresent.MITRALANNULARCALCIFICATIONCalcificationofthemitral“skeleton”UsuallyNOdysfunctionRegurgitationorStenosispossibleF>>M.REGURGITATIONSARRheumaticInfectiousAorticdilatationsSyphilisRheumatoidArthritisMarfanMRMVPInfectiousFen-PhenPapillarymuscles,chordaetendinaeCalcificationofmitralring(annulus).MitralValveProlapse(MVP)MYXOMATOUSdegenerationofthemitralvalveAssociatedwithconnectivetissuedisorders“Floppy”valve3%incidence,F>>MEasilyseenonechocardiogram.MVP:CLINICALFEATURESUsuallyasymptomaticMid-systolic“click”Holosystolicmurmurifregurg.presentOccasionalchestpain,dyspnea97%NOuntowardeffects3%Infectiveendocarditis,mitralinsufficiency,arrythmias,suddendeath..RHEUMATICHeartDiseaseFollowsagroupAstrepinfection,afewweekslaterDECREASEin“developed”countriesPANCARDITIS.ACUTE:-Inflammation-Aschoffbodies-Anitschkowcells-Pancarditis-VegetationsonchordaetendinaeatleafletjunctionCHRONIC:THICKENEDVALVESCOMMISURALFUSIONTHICK,SHORT,CHORDAETENDINAE.CLINICALFEATURESMigratoryPolyarthritisMyocarditisSubcutaneousnodulesErythemamarginatumSydenhamchorea..INFECTIOUSENDOCARDITISMicrobesUsuallystrepviridansOftenStaphaureusinIVDusersEnterococciHA?EK(normaloralflora)HemophilusinfluenzaeActinobacillusCardiobacteriumEikenellaKingellaFungi,rickettsiae,chlamydia.INFECTIOUSENDOCARDITISAcute:50%mortality(course=days)SUB-acute:LOWmortality(course=weeks).VEGETATIONSINFECTIVE>5mmNON-Infective<5mm.DIAGNOSIS=MMm,Mmmm,mmmmmMAJORPositivebloodculture(s)indicatingcharacteristicorganismorpersistenceofunusualorganismEchocardiographicfindings,includingvalve-relatedorimplant-relatedmassorabscess,orpartialseparationofartificialvalveNewvalvularregurgitationminorPredisposingheartlesionorintravenousdruguseFeverVascularlesions,includingarterialpetechiae,subungual/splinterhemorrhages,emboli,septicinfarcts,mycoticaneurysm,intracranialhemorrhage,JanewaylesionsImmunologicphenomena,includingglomerulonephritis,Oslernodes,Rothspots,rheumatoidfactorMicrobiologicevidence,includingsinglecultureshowinguncharacteristicorganismEchocardiographicfindingsconsistentwithbutnotdiagnosticofendocarditis,includingnewvalvularregurgitation,pericarditis..NON-infectiveVEGETATIONS<5mmPETrousseausyndrome(migratorythrombophlebitiswithmalignancies)s/pSwan-GanzLibman-SakswithSLE(bothsidesofvalve).CarcinoidSyndromeEpisodicskinflushingCrampsNausea&VomitingDiarrhea↑serotonin,↑5HIAAinurineFIBROUSINTIMALTHICKENINGRV,Tricuspidvalve,Pulmonicvalve(allRIGHTside)SimilartowhatFen-PhendoesontheLEFTside..ARTIFICIALVALVESMechanicalXenografts(porcine)60%havecomplicationswithin10years.HEARTDISEASECONGENITAL(CHD)?ISCHEMIC(IHD)?HYPERTENSIVE(HHD)?VALVULAR(VHD)?MYOPATHIC(MHD)?PERICARDIALDISEASE.CARDIOMYOPATHIESInflammatoryImmunologicMetabolicDystrophiesGeneticIdiopathicDILATED(DCM)SY-stolicdysfunctionHYPERTROPHIC(HCM)DIA-stolicdysfunctionRESTRICTIVE(RCM)DIA-stolicdysfunction..FunctionalPatternLVEF*

MechanismsofHeartFailureCausesIndirectMyocardialDysfunction(NotCardiomyopathy)Dilated<40%Impairmentofcontractility(systolicdysfunction)Idiopathic;alcohol;peripartum;genetic;myocarditis;hemochromatosis;chronicanemia;doxorubicin(Adriamycin);sarcoidosisIschemicheartdisease;valvularheartdisease;hypertensiveheartdisease;congenitalheartdiseaseHypertrophic50–80%Impairmentofcompliance(diastolicdysfunction)Genetic;Friedreichataxia;storagediseases;infantsofdiabeticmothersHypertensiveheartdisease;aorticstenosisRestrictive45–90%Impairmentofcompliance(diastolicdysfunction)Idiopathic;amyloidosis;radiation-inducedfibrosisPericardialconstriction.CardiacInfectionsVirusesChlamydiaRickettsiaBacteriaFungiProtozoaToxinsAlcoholCobaltCatecholaminesCarbonmonoxideLithiumHydrocarbonsArsenicCyclophosphamideDoxorubicin(Adriamycin)anddaunorubicinMetabolicHyperthroidismHypothyroidismHyperkalemiaHypokalemiaNutritionaldeficiency(protein,thiamine,otheravitaminoses)HemochromatosisNeuromuscularDiseaseFriedreichataxiaMusculardystrophyCongenitalatrophiesStorageDisordersandOtherDepositionsHunter-HurlersyndromeGlycogenstoragediseaseFabrydiseaseAmyloidosisInfiltrativeLeukemiaCarcinomatosisSarcoidosisRadiation-inducedfibrosisImmunologicMyocarditis(severalforms)Post-transplantrejection.DILATEDcardiomyopathyChamberthickness(notjustLVH)AdultsProgressivelydecliningLVEFLVEF~prognosis50%diein2years3MaincausesMyocarditisETOHAdriamycin.Path: 4chamberdilatation Hypertrophy InterstitialFibrosisDCM.ArrhythmogenicRightVentricularCardiomyopathy(ArrhythmogenicRightVentricularDysplasia)ThisisanuncommondilatedcardiomyopathypredominantlyRIGHTventricle.SoisNAXOSsyndrome..HYPERTROPHICcardiomyopathyAlsocalledIHSS,(Idiopathic

Hypertrophic

Subaortic

Stenosis)GENETICdefectsinvolving:Beta-myosinheavychainTroponinTAlpha-tropomyosinMyosinbindingproteinCPATHOLOGY:Massivehypertrophy,Asymmetricseptum,DISARRAYofmyocytes,INTERSTITIALfibrosisCLINICAL:↓chamber