Clinical characteristics and management of coexistent car-diomyopathy in patients with bicuspid aortic valve
Di ZHANG1
Yi-Ting LU1
Ze-Min ZHOU1
Yu-Xiao HU2
Xin-Chang LIU1
Yi QU1
Ya-Xin LIU2
Xian-Liang ZHOU1
1.Department of Cardiology,Fuwai Hospital,National Center for Cardiovascular Diseases,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing,China2.Emergency and Critical Care Center,Fuwai Hospital,National Center for Cardiovascular Diseases,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing,China
摘要：BACKGROUND Bicuspid aortic valve (BAV) is the most common congenital heart disease. However, 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 described. METHODS 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 criteria. Patients with coexist-ent conditions were investigated further. RESULTS Of 3533 patients with BAV screened, 57 (1.6％) had concomitant cardiomyopathy. BAV was combined with HCM in 30 of these patients, with DCM in 19, and with LVNC in eight. Forty-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 LVNC. Heart 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 LVNC. During 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 procedure. There were no deaths or other major adverse cardiovascular events. CONCLUSIONS The prevalence of inherited cardiomyopathy was higher in our patients with BAV than in the general popula-tion. Aortopathy and heart failure were common, with almost half of patients requiring surgery at diagnosis.
机标关键词：characteristicsmanagementclinicalwithaorticbicuspidcar-diomyopathycoexistent
论文发表日期：2023-03-28
在线出版日期：2025-08-15（本平台首次上网日期，不代表文献的发表时间）
页数：9( 205-213 )