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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