肥厚梗阻型心肌病的外科治疗资料讲解

肥厚性梗阻型心肌病

的外科治疗刘晓程泰达国际心血管病医院2009-10-10第二十届长城国际心脏病学会议The20thGreatWallInternationalCongressofCardiology(GW-ICC)常染色体显性遗传疾病发病率1/500A.正常心脏

B.HCM

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

组织学症状体征

动作或药物对HCM的影响心室容积杂音强度左室流出道压力阶差握拳Valsalva动作亚硝酸戊酯异丙肾上腺素β受体阻滞剂苯肾上腺素诊断

Brockenbrough–Braunwald–Morrow征

诊断：心电图

诊断：超声心动图

鉴别诊断

MRIHCM的转归无症状患者房性或室性心律失常

充血性心力衰竭限制性心肌病

终末期HCM

猝死(0.1%-0.7%)心室收缩功能减退

治疗策略药物治疗

β受体阻滞剂

钙离子拮抗剂

丙吡胺（达舒平）

抗心律失常药物

成功率适应证起搏器治疗经皮室间隔消融术指标经皮室间隔酒精消融术室间隔心肌切除术切口腹股沟经皮穿刺胸骨正中切口LVOT压力阶差缓解部分可即时缓解即时成功率(%)>80>95手术死亡率(%)1-20-2恢复时间2-4days1wkLVOT压力阶差变化降至<25mmHg降至<10mmHg术后传导功能异常右束支传导阻滞左束支传导阻滞需安装起搏器比例(%)12-273-10永久起搏器(%)13%2%随访年限(yr)6-830-40外科治疗HCM左室流出道梗阻外科医生年术式Cleland1958经主动脉瓣室间隔心肌切除术Morrow1960经主动脉瓣心肌切开术/切除术Kirklin1961经主动脉瓣/心室心肌切除术Lillihei1963经心房心肌切除术,切开二尖瓣前叶Johnson1964经心房二尖瓣置换术、心肌切除术Cooley1967经右心室心肌切除术Stinson1968心脏移植Cooley1970单纯二尖瓣置换术Rastan,Konno1975主动脉心室成形术Berhard,Cooley1976心尖主动脉管道Vouhe1984经右心室心肌切除术Alvarez-Diaz1984经右心室心肌切除术，补片外科治疗HCM经典Morrow术式HCM的外科治疗改良Morrow术式经典Marrow改良Marrow术后平均压力阶差13-26mmHg5-8mmHg5年内再手术率12%5%主动脉切口联合切口性别男2/女3男5/女4年龄6-62yrs19-77yrs症状晕厥1心绞痛1晕厥1心绞痛2合并症AS1MS1冠心病2IVS-Td18-22mm(20.2±1.48)18mm(6yrspt)20-28mm(25±3.56)压差99-172mmHg(133.6±27.33)86-146mmHg(128.8±24.86)合并症处理AVR1MVR1(Transventricular)CABG4HCM的外科治疗：患者资料主动脉切口联合切口IVS-Td14-18mm(15.6±1.52)12-15mm(13.25±1.26)压力阶差20-40mmHg(29±8.12)4-6mmHg(5.25±0.96)心电图IncompleteLBBB1CompleteLBBB4IncompleteLBBB3CompleteLBBB6两种术式的比较谢谢！SurgeryforHypertrophicCardiomyopathy(HCM)XiaochengLiuTEDAInternationalCardiovascularHospitalChina2009-10-10第二十届长城国际心脏病学会议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