肥厚型心肌病or运动员心脏ppt课件

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1、HypertrophicCardiomyopathy,Athlete’sHeart,orBothACaseofHypertrophicCardiomyopathyRegressionFromCircCardiovascImaging.2015;8:Kebed,MD,MayoClinicCaseAn18-year-oldmanwasreferredforevaluationafteramarkedlyabnormalECGwasdiscoveredataprofessionalhockeyscoutingevent.Hewasot

2、herwisehealthyandasymptomaticHehadnofamilyhistoryofcardiomyopathiesorsuddenunexplaineddeath.ECGUCGAcontrastenhancedtransthoracicechocardiogramshowedapicalHCMwithamaximalLVwallthicknessof24mmandspade-likeappearanceLVchambersizeandsystolicfunctionwerenormalwithoutdynam

3、icobstruction.DiastolicfunctionassessmentshowedindeterminatediastolicfunctiongradeMRItheapexandanteriorseptummeasured25and13mm,Firstpassperfusionwasnormal,butpostcontrastimagesdemonstratedpatchyareasoflategadoliniumenhancementsuggestiveoffibrosisMRIHolterExerciseECGa

4、nd24-hourHoltermonitorshowednoventriculararrhythmiasgenetictestingOngenetictesting,adenovoHCMassociatedmutationinMYH7-encodedβmyosinheavychaingeneatposition530(p.Ile530Val)wasidentified.Thepatient’sparentsand2siblings,allphenotypenegativeforHCM,testednegativeforthemu

5、tation.ThepatientwascounseledonthenaturalhistoryofHCMandtheriskofsuddendeathwithcompetitiveathletics.Noneofthestandardsuddendeathriskmarkerswerepresentattheinitialevaluation,andthereforetherewasnoindicationforanimplantablecardioverterdefibrillator.Thepatientdiscontin

6、uedhisrigorousathletictraining,butcontinuedtoleadarelativelyactivelifestyleHewasseenforfollow-up14monthsaftertheinitialdiagnosis.RepeatcardiacMRIshowedcompleteresolutionoftheLVhypertrophywithmaximalwallthicknessof10mmattheapexHowever,thepatchyareasoflategadoliniumenh

7、ancementwereunchangedOnrepeatnoncontrastechocardiogram,maximumthicknesswas12mmattheapex.TheSTdepressionanddeeplyinvertedTwaveswerelessprominentonhisrepeatECGECGMRIBecauseoftheregressionofhypertrophyafterdetrainingandnegativegenetictestinginfamilymembers,genetictestin

8、gwasrepeatedtoexcludethepossibilityofafalse-positiveresult,buthewasagainfoundtobegenotypepositiveHecontinuedtobeasymptomaticuntil16