肥厚型心肌病患者晕厥发生的机制和处理对策_胡厚源.pptVIP

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:a

50thanniversaryIn1958,DonaldTeare,aforensicpathologistinLondon,reportedeightcasesofsuddendeathcausedby‘‘asymmetricalhypertrophyorbenigntumour’’oftheheartintheBritishHeartJournal.(TeareD.Asymmetricalhypertrophyoftheheartinyoungadults.BrHeartJ.1958;20:1–8.)

HCM病理生理

ComparisonofsurvivalfreefromHCM-relatedcardiacdeath,inpatientswithobstructiveandnon-obstructiveHCM.

SyncopeinhypertrophiccardiomyopathySymptomsofimpairedconsciousness(syncopeandpresyncope)occurin15–25%ofthepatientswithHCM.Syncopetypicallyoccursinyoungerpatientswithsmallerventricles.Impairedconsciousnessisoftenprovokedbyexercise(eitherduringorafter)orbyposturalchange.

MechanismsforsyncopeinHCMArrhythmiaParoxysmalatrialfibrillation/supraventriculartachycardiaCompleteheartblock/sinusnodedysfunctionSustainedventriculartachycardiaPrimaryhaemodynamicmechanismLeftventricularoutflowtractobstruction(LVOTO)AbnormalvascularcontrolmechanismsleadingtoepisodesofhypotensionduetoinappropriatevasodilatationHypotensionduetoimpairedfillingwhenpreloadisreducedinthesettingofdiastolicdysfunction

治疗

AschematicsummaryofthepharmacologictherapyofHCM

为Ⅰa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

Kaplan–Meiersurvivalplotsforall-causecardiacmortalityindisopyramide-treatedandnon–disopyramide-treatedpatientswithHOCM.

ThebeneficialaffectofASAonsymptomsasdeterminedbytheNYHAandtheCCSscoresforheartfailuresymptomsandangina,respectivelyandexercisetoleranceasdeterminedbythetreadmillexercisetime.

ThebeneficialaffectofASAontheresting(diamonds)andprovokedgradients(circles).

Thechangesinseptalthicknessafteralcoholablation

ThechangesinLVejecti