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Prevalence, Characteristics, and Clinical Significance of Concomitant Cardiomyopathies in Subjects with Bicuspid Aortic Valves

 Hyeonju Jeong  ;  Chi Young Shim  ;  Darae Kim  ;  Jah Yeon Choi  ;  Kang-Un Choi  ;  Soo Youn Lee  ;  Geu-Ru Hong  ;  Jong-Won Ha 
 Yonsei Medical Journal, Vol.60(9) : 816-823, 2019 
Journal Title
 Yonsei Medical Journal 
Issue Date
Adult ; Aged ; Aortic Valve/abnormalities* ; Aortic Valve/diagnostic imaging* ; Cardiomyopathy, Hypertrophic/diagnostic imaging ; Cardiomyopathy, Hypertrophic/epidemiology* ; Comorbidity ; Echocardiography ; Female ; Heart Valve Diseases/diagnostic imaging* ; Heart Ventricles/diagnostic imaging* ; Heart Ventricles/physiopathology* ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Phenotype ; Prevalence ; Retrospective Studies ; Ventricular Dysfunction, Left/physiopathology
Bicuspid aortic valve ; dilated cardiomyopathy ; hypertrophic cardiomyopathy ; non-compaction
PURPOSE: The present study aimed to investigate the prevalence, characteristics, and clinical significance of concomitant specific cardiomyopathies in subjects with bicuspid aortic valves (BAVs). MATERIALS AND METHODS: A total of 1186 adults with BAV (850 males, mean age 56±14 years) at a single tertiary center were comprehensively reviewed. Left ventricular non-compaction, hypertrophic cardiomyopathy, and idiopathic dilated cardiomyopathy were confirmed when patients fulfilled current clinical and echocardiographic criteria. Clinical and echocardiographic characteristics, including comorbidities, heart failure presentation, BAV morphology, function, and aorta phenotypes, in BAV subjects with or without specific cardiomyopathies were compared. RESULTS: Overall, 67 subjects (5.6%) had concomitant cardiomyopathies: 40 (3.4%) patients with left ventricular non-compaction, 17 (1.4%) with hypertrophic cardiomyopathy, and 10 (0.8%) with dilated cardiomyopathy. BAV subjects with hypertrophic cardiomyopathy had higher prevalences of diabetes mellitus and heart failure with preserved ejection fraction, and tended to have type 0 phenotype, while BAV subjects with dilated cardiomyopathy showed higher prevalences of chronic kidney disease and heart failure with reduced ejection fraction. BAV subjects with left ventricular non-compaction were significantly younger and predominantly male, and had greater BAV dysfunction and a higher prevalence of normal aorta shape. In multiple regression analysis, cardiomyopathy was independently associated with heart failure (odds ratio 2.795, 95% confidential interval 1.603-4.873, p<0.001) after controlling for confounding factors. CONCLUSION: Concomitant cardiomyopathies were observed in 5.6% of subjects with BAV. A few different clinical and echocardiographic characteristics were found. The presence of cardiomyopathy was independently associated with heart failure.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Shim, Chi Young(심지영) ORCID logo https://orcid.org/0000-0002-6136-0136
Ha, Jong Won(하종원) ORCID logo https://orcid.org/0000-0002-8260-2958
Hong, Geu Ru(홍그루) ORCID logo https://orcid.org/0000-0003-4981-3304
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