HYPERTROPHIC-CARDIOMYOPATHY

HYPERTROPHICCARDIOMYOPATHY•Hypertrophiccardiomyopathyistypicallydefinedasunexplainedventricularhypertrophy,andcanbegenerallydiagnosedinapatientwithhypertrophynotassociatedwithhypertensionorotherobviouscauses,suchasaorticstenosis.Themajorityofcasesofhypertrophic•cardiomyopathyarecausedbysarcomericgeneticmutations,althoughmanyspecificgenemutationshavebeenidentified.Approximately50%ofcasesareautosomaldominant.•Hypertrophiccardiomyopathyexhibitsgreatheterogeneitybothinmorphologicalappearanceandclinicalpresentation.Mostpatientswithhypertrophiccardiomyopathypresentclinicallybetweenages20and40yr.Presentationlaterinlifeisgenerallyassociatedwithlesssevereformsofthedisease.•EchocardiographicFindings•Echocardiographicfeaturesofthemostcommonvariant—assymetricseptalhypertrophy—includesmarkedhypertrophyalongthelengthoftheentireinter-ventricularseptum,oftenattheexpenseoftheleftventricularcavityandnormalorincreasedventricularsystolicfunction.Often,theseptal-to-posteriorwallthicknessratioisgreaterthan1.3。•EchocardiographicFindings•althoughitisalsonotuncommontofindrelativelyconcentrichypertrophy.Typically,hypertrophyextendsdownthemajorityofthelengthoftheseptumandisdistinctfromupperseptaldisproportionatethickeningcommoninhypertensivehypertrophyoftheelderly.•雨雾•雨雾•雨雾NotethemassivehypertrophyconfinedtotheleftventricleandmarkeddilatationofhisleftatrialchamberThepathologicalimages•Systolicanteriormotionofthemitralvalve(SAM).SAMofthemitralvalvedynamicallyobstructsnormalleftventricularoutflow(LVOT).Notetheanteriormovementofbothmitralleaflets(andsupportingmitralstructures)andtheincompletecoaptationatapointhalfwaythebodyoftheelongatedmitralleaflets.TheseresultinthecharacteristicposteriorlydirectedjetofmitralregurgitationM-modeatthemitralvalvelevelcanrevealthetiminganddurationoftheSAMofthemitralvalveleaflets.AlinearrelationshipexistsbetweenthetimeofonsetanddurationofSAM,andtheseverityofthedynamicleftventricularoutflowtract(LVOT)obstruction.Notethatsystolicanteriordisplacementinvolvesnotjusttheanteriormitralleaflet,butalsothechordaeandpapillarymuscles—givingappearanceof“crowding”duringsystoleonM-mode(arrowlabeledSAM)•SystolicAnteriorMotion•Hypertrophiccardiomyopathy•Leftventricularhypertrophy•Infiltrativecardiomyopathieswithseptalinvolvement•Hypercontractilestates•Mechanicalcauses•Others•Two-dimensionalFindings：Asymmetric；isolatedseptalhypertrophy(commonestvariant),althoughconcentrichypertrophyalsocommon.Septal:Posteriorwallthicknessratio1.3elongatedmitralleaflets—coaptationpointalongthebody,notthetipsofLeaflets；•M-mode：SAMofmitralvalve;midsystolicnotching/flutteringoftheaorticvalve；•Dopplerfindings：DynamicLVOTobstruction;“dagger-shaped”，CWprofile;mitralregurgitant(accompanyingSAM)withposteriorly•directedjet;relaxationabnormality/diastolicdysfunctionTheend!ThankYou!WPSOfficeMakePresentationmuchmorefun