Clinical characteristics and management of coexistent cardiomyopathy in patients with bicuspid aortic valve

作     者：Di ZHANG Yi-Ting LU Ze-Min ZHOU Yu-Xiao HU Xin-Chang LIU Yi QU Ya-Xin LIU Xian-Liang ZHOU Di ZHANG;Yi-Ting LU;Ze-Min ZHOU;Yu-Xiao HU;Xin-Chang LIU;Yi QU;Ya-Xin LIU;Xian-Liang ZHOU

作者机构：Department of CardiologyFuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina Emergency and Critical Care CenterFuwai HospitalNational Center for Cardiovascular DiseasesChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina 

出 版 物：《Journal of Geriatric Cardiology》 (老年心脏病学杂志（英文版）)

年 卷 期：2023年第20卷第3期

页      面：205-213页

核心收录：

学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

基　　金：supported by grants from the National Key Research and Development Program of China(2016YFC1300100) National Natural Science Foundation of China(81974042) Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences(2019XK320058 and 2019XK320057) 

主　　题：patients cardiomyopathy aortic 

摘      要：BACKGROUND Bicuspid aortic valve(BAV)is the most common congenital heart ***,the prevalence,clinical characteristics,and current management of BAV associated with inherited cardiomyopathy,including hypertrophic cardiomy-opathy(HCM),dilated cardiomyopathy(DCM),and left ventricular noncompaction(LVNC)have not been well *** Consecutive patients diagnosed with BAV at a large tertiary cardiovascular referral center between 2009 and 2018 were retrospectively assessed for HCM,DCM,and LVNC based on clinical and echocardiographic *** with coexist-ent conditions were investigated *** Of 3533 patients with BAV screened,57(1.6%)had concomitant *** was combined with HCM in 30 of these patients,with DCM in 19,and with LVNC in ***-six patients(80.7%)were male,and the mean age at first dia-gnosis was 47 years for BAV with HCM,49 years for BAV with DCM,and 35 years for BAV with *** failure and aortic valve dysfunction were common in these patients,and the prevalence of coexisting aortopathy was 43.3%,26.3%and 25.0%,re-spectively,for BAV with HCM,DCM and *** the index hospitalization,24 of the 57 patients(42.1%)underwent sur-gery,16(28%)underwent aortic valve and/or aortic surgery,and 16 of the 30 patients with HCM had a Morrow *** were no deaths or other major adverse cardiovascular *** The prevalence of inherited cardiomyopathy was higher in our patients with BAV than in the general *** and heart failure were common,with almost half of patients requiring surgery at diagnosis.