Key result
Concomitant cardiomyopathies were present in 5.6% of adults with bicuspid aortic valves and were independently associated with a significantly higher risk of heart failure (OR 2.795).
Why the study?
The prevalence, characteristics, and clinical significance of concomitant specific cardiomyopathies in subjects with bicuspid aortic valves were unknown.
Observational (n=1,186)
No
Concomitant cardiomyopathies are present in approximately 5.6% of adults with bicuspid aortic valves and are independently associated with a nearly 3-fold increased odds of heart failure.
Concomitant cardiomyopathy in BAV was independently associated with heart failure; supports targeted evaluation but leaves open prospective validation.
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 5614 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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Jeong et al. (2019) conducted an observational in Bicuspid aortic valve (BAV) (n=1,186). Concomitant cardiomyopathy (LVNC, HCM, DCM) vs. Bicuspid aortic valve without cardiomyopathy was evaluated on Heart failure (OR 2.795, 95% CI 1.603-4.873, p=<0.001). Concomitant cardiomyopathies were present in 5.6% of adults with bicuspid aortic valves and were independently associated with a significantly higher risk of heart failure (OR 2.795).
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