Altered aortic shape in bicuspid aortic valve relatives influences blood flow patterns

Abstract

AimsBicuspid aortic valve (BAV) is known to exhibit familial inheritance and is associated with aortopathy and altered aortic haemodynamics. However, it remains unclear whether BAV-related aortopathy can be inherited independently of valve morphology. Methods and resultsFour-dimensional flow magnetic resonance imaging for the in vivo assessment of thoracic aortic 3D blood flow was performed in 24 BAV relatives with trileaflet aortic valves (age ¼ 40 + 14 years) and 15 healthy controls (age ¼ 37 + 10 years). Data analysis included aortic dimensions, shape (round/gothic/cubic), and 3D blood flow characteristics (semiquantitative vortex/helix grading and peak velocities). Cubic and gothic aortic shapes were markedly more prevalent in BAV relatives compared with controls (38 vs. 7%). Ascending aorta (AAo) vortex flow in BAV relatives was significantly increased compared with controls (grading ¼ 1.5 + 1.0 vs. 0.6 + 0.9, P ¼ 0.015). Aortic haemodynamics were influenced by aortic shape: peak velocities were reduced for gothic aortas vs. round aortas (P ¼ 0.003); vortex flow was increased for cubic aortas in the AAo (P , 0.001) and aortic arch (P ¼ 0.004); vortex and helix flows were elevated for gothic aortas in the AAo and descending aorta (P ¼ 0.003, P ¼ 0.029). Logistic regression demonstrated significant associations of shape with severity of vortex flow in AAo (P , 0.001) and aortic arch (P ¼ 0.016) in BAV relatives. ConclusionBAV relatives expressed altered aortic shape and increased vortex flow despite the absence of valvular disease or aortic dilatation. These data suggest a heritable component of BAV-related aortopathy affecting aortic shape and aberrant blood flow, independent of valve morphology.--

Bicuspid aortic valve morphology and its impact on aortic diameters—A systematic review with meta‐analysis and meta‐regression

“…Although the underlying mechanism for reported different disease presentation with different valve phenotypes is still unknown, the simplest hemodynamic theory for aortic BAV aortopathy should not be ruled out. Even though the aortic dilation has been demonstrated in the absence of aortic regurgitation and stenosis and been observed in first degree relatives of BAV patients with normal tricuspid aortic valve, and therefore supporting genetic theory, the hemodynamics seems to be the main mechanism for the development of aortopathy …”

Aortic Valve Stenosis Increases Helical Flow and Flow Complexity: A Study of Intra-Operative Cardiac Vector Flow Imaging

“…The secondary flow in the ascending aorta has been examined primarily in patients with bicuspid aortic valves. This condition seems to enhance the rotation just as aortic valve stenosis does (Hope et al 2011;Meierhofer et al 2013;Schnell et al 2016). Hope et al (2011) stated that helical flow is partly governed by the aortic valve, as a bicuspid valve produces more pronounced helical flow than normal tricuspid valves.…”

4D flow cardiovascular magnetic resonance for monitoring of aortic valve repair in bicuspid aortic valve disease

“…Four-dimensional (4D)-flow cardiovascular magnetic resonance imaging (CMR) has been successfully used to visualize abnormal hemodynamic flow patterns such as helical and vortical flow [15][16][17][18], wall shear stress [19][20][21][22][23], and flow displacement (indicator of outflow asymmetry) [24][25][26][27] in untreated adult congenital heart disease and after aortic valve replacement surgery [28][29][30][31]. We hypothesize that 4D flow CMR might be a comprehensive tool to monitor aortic valve competence and hemodynamic changes after aortic valve repair.…”