Background: In this study, the prevalence, characteristics, and clinical significance of concomitant specific cardiomyopathies (CMs) were investigated in subjects with bicuspid aortic valve (BAV).
Methods: We retrospectively evaluated 1,186 adults with BAV (850 males, mean age 56 ± 14 years). Left ventricular noncompaction (LVNC), hypertrophic CM (HCM), and idiopathic dilated CM (DCM) were diagnosed 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 CM were compared.
Results: Overall, 67 subjects (5.6 %) had concomitant CMs: 40 (3.4%) patients with LVNC, 17 (1.4%) with HCM, and 10 (0.8%) with DCM. BAV subjects with HCM had a higher prevalence of diabetes mellitus, heart failure with preserved ejection fraction (HFPEF), and tended to have type 0 phenotype, while BAV subjects with DCM showed a higher prevalence of chronic kidney disease and heart failure with reduced ejection fraction (HFREF). BAV subjects with LVNC were significantly younger, predominantly male, and had greater BAV dysfunction and a higher prevalence of normal aorta shape. In multiple regression analysis, presence of CM was independently associated with heart failure (odds ratio (OR) 2.866, 95% confidential interval (CI) 1.652–4.974, p < 0.001) even after controlling confounding factors.
Conclusion: Concomitant CMs were observed in 5.6% of subjects with BAV. A few clinical and echocardiographic characteristics including comorbidities, heart failure presentation, BAV morphology, function, and presence of aortopathy were found. The presence of CM was independently associated with heart failure.
Ebstein's Anomaly With Left Ventricular Noncompaction and Bicuspid Aortic Valve