hypertrophic-cardiomyopathy肥厚型心肌病

(我是医生)2014年9月9日刘辉影像医学部放射科2019/10/24210984723•病史•男，66岁，活动后气促伴胸闷一年余，加重7天影像医学部放射科2019/10/243T2WIT2WI/STIRPSIR影像医学部放射科2019/10/244T2WIT2WI/STIRPSIR影像医学部放射科2019/10/245B-TFEPSIR影像医学部放射科问题•CMR表现？•可能的诊断？•CMR图像上应该注意观察的内容？2019/10/246影像医学部放射科CMR评价肥厚型心肌病（HCM）•acommongeneticcardiovasculardisease•aprevalenceofLVhypertrophyinthegeneralpopulation：0.2%2019/10/247MaronBJ,etal.Circulation.1995;92:785–9影像医学部放射科Clinicaldiagnosis•Hypertrophiedandnondilatedleftventricle(LV;wallthickness≥15)•absenceofanothercardiacorsystemicdiseasecapableofproducingsimilarmagnitudeofhypertrophy2019/10/248GershBJ,etal.2011ACCF/AHA….JAmCollCardiol/Circulation.2011;58:212–260影像医学部放射科Genetictest•anautosomaldominantmutation•8genesareknowntodefinitivelycauseHCM•identifythepathogenicHCMmutation–60%-70%ofpatientswithapositivefamilyhistory–10%-50%ofpatientswithoutafamilyhistory2019/10/249MoritaH,etal.NEnglJMed.2008;358:1899–908影像医学部放射科Prognosis/RiskStratification2019/10/2410Maron,BJetal.JAmCollCardiol2003,42:1687-1713.影像医学部放射科Prognosis/RiskStratification•LVOTobstruction•LVthickness•lategadoliniumenhancement(LGE)2019/10/2411影像医学部放射科LVOTobstruction2019/10/241210633773Elongatedanteriormitralvalve≥30mmMaron,MS.JCardiovascMagnReson.2012Feb1;14:13.doi:10.1186/1532-429X-14-13影像医学部放射科LGE2019/10/2413影像医学部放射科Multicenterstudy:significanceofLGEinHCM•7majorHCMcenters•2001-2010•1293consecutivepatients•Studyentry:CMR•Follow-up:3.4±1.7years•SuddenDeathevent:37patients(3%)2019/10/2414Chanetal.Circulation.2014;130:484-495影像医学部放射科Multicenterstudy:significanceofLGEinHCM•LGEwaspresentin548patients:42%•TheextentofLGE:–29%:≤10%–7%:11%-19%–6%:≥20%2019/10/2415Chanetal.Circulation.2014;130:484-495影像医学部放射科2019/10/2416Chanetal.Circulation.2014;130:484-495影像医学部放射科2019/10/2417Chanetal.Circulation.2014;130:484-495高危可能发生SCD患者：ICD置入进行预防性治疗精准医疗：通过定量化的方法发现高危患者进行早期干预影像医学部放射科Differentialdiagnosis•hypertensiveheartdisease•athlete’sheart:–Usuallymaximumwallthickness:13to15mm•metabolicdisorderswithLVhypertrophy–Danondisease•infiltrativestoragedisorderswithLVhypertrophy–sarcodosis2019/10/2418MahrholdtHetal.EurHeartJ2005;26:1461-1474影像医学部放射科HCM•Patchyenhancement•RVinsertionwithLV2019/10/2419影像医学部放射科•48yearoldmanwithrecentdiagnosisofpulmonarysarcoid•FromBWH2019/10/2420影像医学部放射科Summary•DiagnosisforHCM–Clinicaldiagnosis–Genetictest•Prognosis/RiskStratification–QuantitiveLGE:15%•Differentialdiagnosis2019/10/2421