肥厚型心肌病患者晕厥发生的机制和处理对策胡厚源.ppt

肥厚型心肌病患者晕厥发生的机制和处理对策,第三军医大学西南医院心内科胡厚源,Hypertrophiccardiomyopathy:a50thanniversary,In1958,DonaldTeare,aforensicpathologistinLondon,reportedeightcasesofsuddendeathcausedbyasymmetricalhypertrophyorbenigntumouroftheheartintheBritishHeartJournal.(TeareD.Asymmetricalhypertrophyoftheheartinyoungadults.BrHeartJ.1958;20:18.),HCM病理生理,ComparisonofsurvivalfreefromHCM-relatedcardiacdeath,inpatientswithobstructiveandnon-obstructiveHCM.,Syncopeinhypertrophiccardiomyopathy,Symptomsofimpairedconsciousness(syncopeandpresyncope)occurin1525%ofthepatientswithHCM.Syncopetypicallyoccursinyoungerpatientswithsmallerventricles.Impairedconsciousnessisoftenprovokedbyexercise(eitherduringorafter)orbyposturalchange.,MechanismsforsyncopeinHCM,ArrhythmiaParoxysmalatrialfibrillation/supraventriculartachycardiaCompleteheartblock/sinusnodedysfunctionSustainedventriculartachycardiaPrimaryhaemodynamicmechanismLeftventricularoutflowtractobstruction(LVOTO)AbnormalvascularcontrolmechanismsleadingtoepisodesofhypotensionduetoinappropriatevasodilatationHypotensionduetoimpairedfillingwhenpreloadisreducedinthesettingofdiastolicdysfunction,治疗,猝死高危的患者植入ICD，提高生存率：持续性VT胺碘酮或ICD非持续性VT，无SCD危险因素胺碘酮非持续性VT，伴有SCD危险因素ICD阵发性AF胺碘酮；控制AF患者的HR阻滞剂、维拉帕米；心动过缓起搏器植入（DDD）；减轻LVOT梗阻药物、室间隔酒精消融术(Alcoholseptalablation,ASA)、肥厚室间隔切开/切除术（myectomy）、心脏移植术。,AschematicsummaryofthepharmacologictherapyofHCM,丙吡胺（disopyramide）,为a类抗心律失常药；也是另一种负性肌力药，负性肌力作用强，可用于治疗HOCM；除抗心律失常外，还能显著改善血流动力学。,LVOTGbeforeandaftertheintravenousadministrationofdisopyramidetoapatientwithHOCM,KaplanMeiersurvivalplotsforall-causecardiacmortalityindisopyramide-treatedandnondisopyramide-treatedpatientswithHOCM.,丙吡胺的注意事项,应监测QT间期延长；丙吡胺并不能改善HCM的舒张功能；对有发生房颤可能的患者有益处；副作用包括抗胆碱能作用，如口干、眼干、便秘、排尿困难。,室间隔心肌酒精消融术Alcoholseptalablation,ASA,ThebeneficialaffectofASAonsymptomsasdeterminedbytheNYHAandtheCCSscoresforheartfailuresymptomsandangina,respectivelyandexercisetoleranceasdeterminedbythetreadmillexercisetime.,ThebeneficialaffectofASAontheresting(diamonds)andprovokedgradients(circles).,Thechangesinseptalthicknessafteralcoholablation,ThechangesinLVejectionafteralcoholablati