肥厚梗阻型心肌病的外科治療資料講解

肥厚性梗阻型心肌病

的外科治療劉曉程泰達(dá)國(guó)際心血管病醫(yī)院2009-10-10第二十屆長(zhǎng)城國(guó)際心臟病學(xué)會(huì)議The20thGreatWallInternationalCongressofCardiology(GW-ICC)常染色體顯性遺傳疾病發(fā)病率1/500A.正常心臟

B.HCM

C.HCM（馬森三色染色法）（ImagescourtesyofRobertPadera,MDBrighamandWomen’sHospitalDepartmentofPathology,Boston,MA.）

組織學(xué)癥狀體征

動(dòng)作或藥物對(duì)HCM的影響心室容積雜音強(qiáng)度左室流出道壓力階差握拳Valsalva動(dòng)作亞硝酸戊酯異丙腎上腺素β受體阻滯劑苯腎上腺素診斷

Brockenbrough–Braunwald–Morrow征

診斷：心電圖

診斷：超聲心動(dòng)圖

鑒別診斷

MRIHCM的轉(zhuǎn)歸無(wú)癥狀患者房性或室性心律失常

充血性心力衰竭限制性心肌病

終末期HCM

猝死(0.1%-0.7%)心室收縮功能減退

治療策略藥物治療

β受體阻滯劑

鈣離子拮抗劑

丙吡胺（達(dá)舒平）

抗心律失常藥物

成功率適應(yīng)證起搏器治療經(jīng)皮室間隔消融術(shù)指標(biāo)經(jīng)皮室間隔酒精消融術(shù)室間隔心肌切除術(shù)切口腹股溝經(jīng)皮穿刺胸骨正中切口LVOT壓力階差緩解部分可即時(shí)緩解即時(shí)成功率(%)>80>95手術(shù)死亡率(%)1-20-2恢復(fù)時(shí)間2-4days1wkLVOT壓力階差變化降至<25mmHg降至<10mmHg術(shù)后傳導(dǎo)功能異常右束支傳導(dǎo)阻滯左束支傳導(dǎo)阻滯需安裝起搏器比例(%)12-273-10永久起搏器(%)13%2%隨訪年限(yr)6-830-40外科治療HCM左室流出道梗阻外科醫(yī)生年術(shù)式Cleland1958經(jīng)主動(dòng)脈瓣室間隔心肌切除術(shù)Morrow1960經(jīng)主動(dòng)脈瓣心肌切開(kāi)術(shù)/切除術(shù)Kirklin1961經(jīng)主動(dòng)脈瓣/心室心肌切除術(shù)Lillihei1963經(jīng)心房心肌切除術(shù),切開(kāi)二尖瓣前葉Johnson1964經(jīng)心房二尖瓣置換術(shù)、心肌切除術(shù)Cooley1967經(jīng)右心室心肌切除術(shù)Stinson1968心臟移植Cooley1970單純二尖瓣置換術(shù)Rastan,Konno1975主動(dòng)脈心室成形術(shù)Berhard,Cooley1976心尖主動(dòng)脈管道Vouhe1984經(jīng)右心室心肌切除術(shù)Alvarez-Diaz1984經(jīng)右心室心肌切除術(shù)，補(bǔ)片外科治療HCM經(jīng)典Morrow術(shù)式HCM的外科治療改良Morrow術(shù)式經(jīng)典Marrow改良Marrow術(shù)后平均壓力階差13-26mmHg5-8mmHg5年內(nèi)再手術(shù)率12%5%主動(dòng)脈切口聯(lián)合切口性別男2/女3男5/女4年齡6-62yrs19-77yrs癥狀暈厥1心絞痛1暈厥1心絞痛2合并癥AS1MS1冠心病2IVS-Td18-22mm(20.2±1.48)18mm(6yrspt)20-28mm(25±3.56)壓差99-172mmHg(133.6±27.33)86-146mmHg(128.8±24.86)合并癥處理AVR1MVR1(Transventricular)CABG4HCM的外科治療：患者資料主動(dòng)脈切口聯(lián)合切口IVS-Td14-18mm(15.6±1.52)12-15mm(13.25±1.26)壓力階差20-40mmHg(29±8.12)4-6mmHg(5.25±0.96)心電圖IncompleteLBBB1CompleteLBBB4IncompleteLBBB3CompleteLBBB6兩種術(shù)式的比較謝謝！SurgeryforHypertrophicCardiomyopathy(HCM)XiaochengLiuTEDAInternationalCardiovascularHospitalChina2009-10-10第二十屆長(zhǎng)城國(guó)際心臟病學(xué)會(huì)議The20thGreatWallInternationalCongressofCardiology(GW-ICC)Aautosomaldominantdisease.MorbidityofHCMis1per500.LocusNameGeneSymbolProteinNameOMIM%ofHCMCausedbyMutationsinThisGeneMolecularGeneticTestAvailabilityforHMC1AllelicDisorders2CMH1MYH7Myosinheavychain,cardiacmusclebetaisoform160760

19260040%ClinicalDCM3,LaingdistalmyopathyCMH4MYBPC3Myosin-bindingproteinC,cardiac-type60095840%ClinicalDCMCMH2TNNT2TroponinT,cardiacmuscle1151955%ClinicalDCMCMH7TNNI3TroponinI,cardiacmuscle1910445%ClinicalDCM,restrictivecardiomyopathyCMH3TPM1Tropomyosin1alphachain115196

1910102%ClinicalDCMCMH10MYL2Myosinregulatorylightchain2,ventricular/cardiacmuscleisoform160781

608758UnknownClinicalCMH8MYL3Myosinlightpolypeptide3160790

6087511%ClinicalACTC1Actin,alphacardiacmuscle1102540UnknownClinicalDCMCSRP3Cysteineandglycine-richprotein3,muscleLIMprotein600824UnknownResearchCMH9TTNTitin188840ClinicalDCM,UdddistalmyopathyMYH6Myosinheavychain,cardiacmusclealphaisoform160710ResearchDCMTCAPTelothonin604488ResearchLGMD2G4,DCMOthergenesimplicatedinHCM5TNNC1TroponinC,slowskeletalandcardiacmuscles191040UnknownClinicalDCM1.PertheGeneTestsLaboratoryDirectory2.Allelicdisorders=otherphenotypescausedbymutationinthesamegene3.DCM=dilatedcardiomyopathy4.LGMD=limb-girdlemusculardystrophy5.TheconsensusoftheGeneReviewauthorsisthatadditionalconfirmatorydatasupportingpathogenicityforthisgeneisnecessary.A.Normalheart

B.HCM

C.HCMwithMassontrichromestaining（ImagescourtesyofRobertPadera,MDBrighamandWomen’sHospitalDepartmentofPathology,Boston,MA.）

HISTOLOGYsymptomssigns

EffectsofPhysiologicandPharmacologicManeuversonHCMManeuverVentricularVolumeMurmurIntensityLVOTGradientHandgripValsalvaAmylnitriteIsoproterenolBetablockerPhenylephrineDIAGNOSIS

Brockenbrough–Braunwald–MorrowSign

DIAGNOSIS:ECG

DIAGNOSIS:UCG

UCGfordifferentialDiagnosis

MRIPROGNOSISOFHCMNoormildsymptomsArrhythmia

Congestiveheartfailurerestrictivecardiomyopathy

End-stageHCM

Suddendeath(0.1%-0.7%)systolicleftventriculardysfunction

StrategyofTreatmentMedicalmanagement

Betablockers

CalciumchannelblockersDisopyramideAntiarrhythmics

SuccessrateIndicationPacemakerTherapyPercutaneousseptalablationParameterPercutaneousAlcoholSeptalAblationSurgicalMyectomyInvasivenessPercutaneousgroinaccessSternotomyOnsetofreductioninLVOTgradientSomedecreaseingradientinstantly,but6-12moforfulleffectInstantaneousSuccessrate(%)>80>95Proceduralmortality(%)1-20-2Recoverytime2-4days1wkEffectonLVOTgradientDecreasesto<25mmHgDecreasesto<10mmHgPostprocedureconductionabnormalityRightbundlebranchblockLeftbundlebranchblockNeedforpermanentpacemaker—allpatients(%)12-273-10Needforpermanentpacemakerifnopreexistingconductionabnormalities(%)13%2%Lengthoffollow-up(yr)6-830-40SurgicaltechniquesforoutflowobstructioninpatientswithHCMSurgeonYearProcedureCleland1958TransaorticseptalmyectomyMorrow1960Transaorticmyotomy/myectomyKirklin1961Transaortic/transventricularmyectomyLillihei1963Transatrialmyectomy,detachmentmitralvalveJohnson1964Transatrialmitralvalvereplacement,myectomyCooley1967Trans-rightventricularseptalmyectomyStinson1968CardiactransplantationCooley1970MitralvalvereplacementwithoutmyectomyRastan,Konno1975AortoventriculoplastyBerhard,Cooley1976ApicoaorticconduitVouhe1984Trans-rightventricularmyectomyAlvarez-Diaz1984Trans-rightventricularmyectomy,patchSurgeryforHCMClassicalMorrowProcedureSurgeryforHCMModifiedMorrowProcedureClassicalMorrowProcedureModifiedMorrowProcedureMeanpressuregradientpost-op.13-26mmHg5-8mmHgRe-op.rat