hypertrophic cardiomyopathy肥厚型心肌病.pptxVIP

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hypertrophic cardiomyopathy肥厚型心肌病

2014年9月9日刘史男,66岁,活动后气促伴胸闷一年余,加重7天T2WIT2WI/STIRPSIRT2WIT2WI/STIRPSIRB-TFEPSIRCMR评价肥厚型心肌病(HCM)Maron BJ, et al. Circulation. 1995;92:785–9a common genetic cardiovascular diseasea prevalence of LV hypertrophy in the general population: 0.2%Clinical diagnosisHypertrophied and nondilated left ventricle (LV; wall thickness ≥15) absence of another cardiac or systemic disease capable of producing similar magnitude of hypertrophyGersh BJ, et al. 2011 ACCF/AHA …. J Am Coll Cardiol/Circulation. 2011;58:212–260Genetic testan autosomal dominant mutation8 genes are known to definitively cause HCMidentify the pathogenic HCM mutation 60% - 70% of patients with a positive family history10% - 50% of patients without a family historyMorita H, et al. N Engl J Med. 2008;358:1899 –908Prognosis/Risk Stratification Maron, BJ et al. J Am Coll Cardiol 2003,42:1687-1713.Prognosis/Risk Stratification LVOT obstructionLV thicknesslate gadolinium enhancement (LGE) LVOT obstructiolongated anterior mitral valve≥30 mmMaron, MS. J Cardiovasc Magn Reson.? 2012 Feb 1;14:13. doi: 10.1186/1532-429X-14-13?LGEMulticenter study:significance of LGE in HCM7 major HCM centers2001consecutive patientsStudy entry:CMRFollow-up:3.4±1.7 yearsSudden Death event:37 patients (3%)Chan et al.Circulation. 2014;130:484-495Multicenter study:significance of LGE in HCMLGE was present in 548 patients: 42%The extent of LGE:29%: ≤10% 7%: 11%-19%6%: ≥20%Chan et al.Circulation. 2014;130:484-495Chan et al.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Chan et al.Circulation. 2014;130:484-495Differential diagnosishypertensive heart disease athlete’s heart: Usually maximum wall thickness: 13 to 15 mmmetabolic disorders with LV hypertrophyDanon diseaseinfiltrative storage disorders with LV hypertrophy sarcodosisMahrholdt H et al. Eur Heart J 2005;26:1461-1474HCMPatchy enhancementRV insertion with LV48 year old man with recent diagnosis of pulmonary sarcoidFrom BWHSummaryDi

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