Bicuspid aortic valve: prompt identification of high-risk groups in a heterogeneous disease

Abstract

Abstract Background Bicuspid aortic valve (BAV) is a congenital heart disease that can be associated with aortic and/or valve complications. Although most BAV are predisposed to progressive calcification, little is known on variables related to significant valvular dysfunction. BAV is a heterogeneous disease, therefore it is important to identify high-risk groups for a closer follow- up and timely intervention. Objective Identify the main determinants of primary cardiovascular events (aortic valve replacement, aortic surgery and death) in a cohort of adults with BAV. Methods We included 325 consecutive patients with non-syndromatic BAV (2010–2019) referred to our hospital (3rd level). Clinical information was prospectively collected. All patients underwent serial echocardiograms and Cardiac CT was available in 125 patients (38%). We performed univariate and multivariate analyses and Kaplan-Meyer survival analysis with log-rank test. A p-value <0.05 was considered significant. Results 325 patients (44.4±15.3 years, 73% men), right–left coronary cusp fusion (RL) in 79%,and presence of raphe in 77%. 100 patients (31.2%) had significant aortic valve dysfunction and 103 (31.7%) had aortic dialatation (>40mm) at the onset of the follow-up. Twenty-nine patients required cardiac surgery (8.9%, mainly symptomatic severe aortic stenosis with aortic valve replacement)and 3 died (1%) during a follow-up of 6.3±1.2 years. Patients with at least 2 of the following determinants at baseline: aortic valve prolapse, aortic valve calcification >1, age >50 years and/or aorta >45mm had higher risk of major cardiovascular events during follow-up (Chi2 for log rank test=27,229 p=0,000005). Conclusions In this study population of young adults with BAV we observed a high incidence of events related to the BAV. Age>50 years, aortic valve calcification, aortic valve prolapse and aortic dilatation were independently associated with primary cardiac events. Despite significant BAV heterogeneity, the identification of risk factors may help to stratify the risk of valvular dysfunction, aortic dilatation and major cardiovascular events. BAV: Risk factors for cardiac surgery Funding Acknowledgement Type of funding source: None