肥厚性心肌病的最新指南

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1、2011ACCF/AHAGuidelinefortheDiagnosisandTreatmentofHypertrophicCardiomyopathyTheSecondAffiliatedHospitalofNanjingMedicalUniversityTheSecondClinicalMedicalSchoolofNanjingMedicalUniversity南京医科大学第二附属医院——肥厚型心肌病最新指南解读先证者之兄先证者之二姐先证者之大姐先证者先证者之弟先证者之长子先证者之次子先证者之父先证者之母肥厚性心肌病—家系图先证者心电图图4心脏四腔心平面图5心脏

2、三腔心平面提示左心室心肌壁明显增厚先证者心脏MRI先证者之兄：男性，58岁心肌病心肌病是指各种病因引起的一组非均质的心肌病变，以心肌损害为特征，包括心脏机械和电活动的异常，伴有心功能障碍的心脏疾病，病因多种多样。DefinitionandClassificationofCardiomyopathy1980s1995WHO/ISFC2006AHAContemporaryClassification2006ScientistsConsensusonCardiomyopathyinChina2008ESCClassification心肌病心肌病分类Ⅰ原发性心肌病及继发性心肌

3、病原发性心肌病(Primarycardiomyopathies)病变仅局限于心脏的心肌。继发性心肌病(Secondarycardiomyopathies)心肌病病变是全身多器官病变的一部分。酒精性心肌病药物性心肌病围生期心肌病代谢性心肌病免疫性心肌病心脏瓣膜病致心肌病其他继发性心肌病变非缺血性心肌病http://my.clevelandclinic.org/heart/disorders/heartfailure/cardiomyopathy.aspxHCMisageneticdiseaseThereareatleast2millionsHCMpatientsinCh

4、inaItwillbemoreoftentoseeinelderpatientsThemostnewguidelineforHCMfromACC/AHA肥厚型心肌病11个-明确；11-个别报告，23个，NEXN肥厚型心肌病：遗传病，先心病JACC和Circulation同时发表肥厚型心肌病的指南，总共57页。是2003年AHA/ACC有关HCM，2006年心肌病分类，2008年欧洲心肌病共识后的有关心肌病重要阐述。从HCM发病、临床分类、诊断、治疗，每一个方面均提出了建议。2011ACCF/AHAGuidelinefortheDiagnosisandTreatment

5、ofHypertrophicCardiomyopathy肥厚型心肌病2011ACCF/AHAGuidelinefortheDiagnosisandTreatmentofHypertrophicCardiomyopathyDevelopedinCollaborationwiththeAmericanAssociationforThoracicSurgery,AmericanSocietyofEchocardiography,AmericanSocietyofNuclearCardiology,HeartFailureSocietyofAmerica,HeartRhyth

6、mSociety,SocietyforCardiovascularAngiographyandInterventions,andSocietyofThoracicSurgeons©AmericanCollegeofCardiologyFoundationandAmericanHeartAssociation,Inc.BernardJ.Gersh,MB,ChB,DPhil,FACC,FAHA,Co-ChairBarryJ.Maron,MD,FACC,Co-ChairClassificationofRecommendationsandLevelsofEvidenceAre

7、commendationwithLevelofEvidenceBorCdoesnotimplythattherecommendationisweak.Manyimportantclinicalquestionsaddressedintheguidelinesdonotlendthemselvestoclinicaltrials.Althoughrandomizedtrialsareunavailable,theremaybeaveryclearclinicalconsensusthataparticulartestortherapyisusefulo