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

Abstract

24Background: In this study, the prevalence, characteristics, and clinical significance of 25 concomitant specific cardiomyopathies were investigated in subjects with bicuspid aortic 26 valve (BAV). 27 Methods: We retrospectively evaluated 1,186 adults with BAV (850 males, mean age 56 ± 28 14 years). Left ventricular non-compaction, hypertrophic cardiomyopathy, and idiopathic 29 dilated cardiomyopathy were diagnosed when patients fulfilled current clinical and 30 echocardiographic criteria. Clinical and echocardiographic characteristics including 31 comorbidities, heart failure presentation, BAV morphology, function, and aorta phenotypes in 32 BAV subjects with or without specific cardiomyopathies were compared. 33 Results: Overall, 67 subjects (5.6 %) had concomitant cardiomyopathies: 40 (3.4%) patients 34 with left ventricular non-compaction, 17 (1.4%) with hypertrophic cardiomyopathy, and 10 35 (0.8%) with dilated cardiomyopathy. BAV subjects with hypertrophic cardiomyopathy had a 36higher prevalence of diabetes mellitus, heart failure with preserved ejection fraction, and 37 tended to have type 0 phenotype, while BAV subjects with dilated cardiomyopathy showed a 38 higher prevalence of chronic kidney disease and heart failure with reduced ejection fraction. 39BAV subjects with left ventricular non-compaction were significantly younger, 40 predominantly male, and had greater BAV dysfunction and a higher prevalence of normal 41 aorta shape. In multiple regression analysis, presence of cardiomyopathy was independently 42 associated with heart failure (odds ratio 2.866, 95% confidential interval 1.652-4.974, p < 43 0.001) even after controlling confounding factors. 44 Conclusion: Concomitant cardiomyopathies were observed in 5.6% of subjects with BAV. A 45 few clinical and echocardiographic characteristics including comorbidities, heart failure 46 presentation, BAV morphology, function, and presence of aortopathy were found. The 3 47 presence of cardiomyopathy was independently associated with heart failure. 48 Keywords: Bicuspid aortic valve, left ventricular non-compaction, hypertrophic 49 cardiomyopathy, dilated cardiomyopathy 50 51 Abbreviations 52 BAV = bicuspid aortic valve 53 LVNC = left ventricular non-compaction 54 CM = cardiomyopathy 55 HCM = hypertrophic cardiomyopathy 56 DCM = dilated cardiomyopathy 57 LVEF = left ventricular ejection fraction 58 OR = odds ratio 59 CI = confidence interval 60 61 62 63 64 65 66 67 4 68 131 failure with preserved ejection fraction (LVEF ≥ 50%) and heart failure with reduced ejection 132 fraction (LVEF < 50%). 133 Continuous variables were expressed as mean ± standard deviation (SD). Categorical 134 variables were expressed as number (percentage). Comparisons between the groups were 135 performed using standard λ 2 tests for categorical variables and paired t-tests for continuous 136 variables. Univariate and multivariate logistic regression analyses were performed. All 137 statistical analyses were performed using SPSS Statistics, software version 23.0 (IBM, 138 Armonk,...