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OBJECTIVES A bicuspid aortic valve (BAV) is characterized by variable phenotypic manifestations, as well as longitudinal evolution of valve dysfunction and ascending aorta dilatation. The present study investigated the impact of severe aortic stenosis (AS) on the flow patterns and wall shear stress (WSS) distribution in BAV patients with right–left (RL) and right-non-coronary (RN) cusp fusion types, and the study aimed to reveal whether aortic dysfunction could further alter intrinsic aortic haemodynamic aberrations generated by abnormal BAV cusp fusion patterns. METHODS Four-dimensional flow magnetic resonance imaging was performed in 120 BAV subjects and 20 tricuspid aortic valve controls. BAV patients were evenly categorized into 4 cohorts, including RL and RN BAV with no more than mild aortic dysfunction as well as RL and RN BAV-AS with isolated severe AS. RESULTS BAV subjects exhibited eccentric outflow jets resulting in regional WSS elevation at the right-anterior position of the ascending aorta in the RL group and the right-posterior location in the RN group (P &lt; 0.005). The presence of severe AS resulted in accelerated outflow jets and more prominent flow and WSS eccentricity (P &lt; 0.005) by marked helical (P = 0.014) and vortical flow formation (P &lt; 0.005), as well as increased prevalence of tubular and transverse arch dilatation. The changes to the flow jet in BAV-AS subjects blurred the differences in peak flow velocity and WSS distribution between RL and RN BAV. Differences in the phenotypes of aortopathy were associated with changes in functional haemodynamic parameters such as flow displacement and WSS eccentricity. CONCLUSIONS Severe AS markedly exacerbated aortic flow aberrations in BAV patients and masked the existing distinct flow features deriving from RL and RN fusion types. Longitudinal studies are needed to investigate the evolution of ascending aortic dilatation relative to the interaction between intrinsic cusp fusion types and acquired severe valve dysfunction.

Aortic stenosis exacerbates flow aberrations related to the bicuspid aortic valve fusion pattern and the aortopathy phenotype