肥厚型心肌病患者晕厥发生的机制和处理对策-胡厚源

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1、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:a50thanniversaryIn1958,DonaldTeare,aforensicpathologistinLondon,reportedeightcasesofsuddendeathcausedby‘‘asymmetricalhypertrophyorbenigntumour’’oftheheartintheBritishHeartJournal.(TeareD.Asymmetricalhypertrophyoft

2、heheartinyoungadults.BrHeartJ.1958;20:1–8.)HCM病理生理ComparisonofsurvivalfreefromHCM-relatedcardiacdeath,inpatientswithobstructiveandnon-obstructiveHCM.SyncopeinhypertrophiccardiomyopathySymptomsofimpairedconsciousness(syncopeandpresyncope)occurin15–25%ofthepatientswithHCM.Syncope

3、typicallyoccursinyoungerpatientswithsmallerventricles.Impairedconsciousnessisoftenprovokedbyexercise(eitherduringorafter)orbyposturalchange.MechanismsforsyncopeinHCMArrhythmiaParoxysmalatrialfibrillation/supraventriculartachycardiaCompleteheartblock/sinusnodedysfunctionSustaine

4、dventriculartachycardiaPrimaryhaemodynamicmechanismLeftventricularoutflowtractobstruction(LVOTO)AbnormalvascularcontrolmechanismsleadingtoepisodesofhypotensionduetoinappropriatevasodilatationHypotensionduetoimpairedfillingwhenpreloadisreducedinthesettingofdiastolicdysfunction治疗

5、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－β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Ⅰa类抗心律失常药；也是另一种负性肌力

6、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。LVOTGbeforeandaftertheintravenousadministrationofdisopyramidetoapatientwithHOCMKaplan–Meiersurvivalplotsforall-causecardiacmortalityindisopyramide-treatedandnon–disopyramide-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

7、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,ASAThebeneficialaffectofASAonsymptomsasdeterminedbytheNYHAandtheCCSscoresforheartfailuresymptomsandangina,respectivelyandexercisetoleranceasdeterminedbythetreadmillexercisetime.ThebeneficialaffectofASAontheres

8、ting(diamonds)andprovokedgradients(circles).Thechanges