Aortic Arch Tortuosity, a Novel Biomarker for Thoracic Aortic Disease, is increased in Adults with Bicuspid Aortic Valve
International Journal of Cardiology , 2019
Introduction: Arterial tortuosity has emerged as a predictor ofadverse outcomes in congenital aor... more Introduction: Arterial tortuosity has emerged as a predictor ofadverse outcomes in congenital aortopathies using 3D reconstructed images. We validated a new method to estimate aortic arch tortuosity on 2D CT. We hypothe- size that arch tortuosity may identify bicuspid aortic valve (BAV) patients at high risk to develop thoracic aortic aneurysms or aortic dissections (TAD). Methods: BAV subjects with chest CT scans were retrospectively identified in our clinical records and matched to tricuspid aortic valve (TAV) controls by age, gender, and presentation with TAD. Subjects with prior ascending aortic intervention were excluded. Measurements included aortic arch tortuosity, length, angle, width and height. Total aortic tortuosity was estimated in subjects with available abdominal images. Results: 120 BAV and 234 TAV subjects were included. Our 2D measurements were highly correlated with 3D midline arch measurements and had high inter- and intra-observer reliability. Compared to TAV, BAV subjects had increased arch tortuosity (median 1.76 [Q1-Q3: 1.62–1.95] vs. 1.63 [1.53–1.78], P b 0.01), length (149 [136–160] vs. 135 [122–152] mm, P b 0.01), height (46 [41–53] vs. 39 [34–47] mm, P b 0.01), and vertex acuity (70 [61–77] vs. 75 [68–81] degree, P b 0.01). In a multivariable analysis, arch tortuosity remained independently associated with BAV after adjusting for aortic diameter and other clinical characteristics. Conclusions: We found that aortic arch tortuosity is significantly increased in BAV and may identify BAV patients who are at increased risk for TAD. Further studies to evaluate the association between tortuosity and clinical out- comes are in progress.
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Papers by Andrea Guala
Methods: Seventy-six consecutive patients with BAVs (mean age, 53 6 15 years; 65% men) who underwent both TTE and MRI for ascending aortic assessment in a follow-up protocol were included in the study. Maximum aortic root and ascending aortic diameters were compared.
Results: For the whole population, TTE slightly underestimated aortic root diameter (difference, ?0.8 6 2.9 mm; P = .02). However, agreement was significantly better in BAV with fusion of the left and right coronary cusps than with fusion of the right coronary and noncoronary cusps, both with (type 1) and without (type 0) raphe (mean difference, 0.1 6 2.5 vs ?2.8 6 2.8 mm, P < .001, respec- tively). In raphe BAV, mean absolute differences of maximum diameters between both techniques were significantly greater in asymmetric versus symmetric aortic roots (3.3 6 2.2 vs 1.6 6 1.9 mm, P = .002). BAV type and root asymmetry were independent related to measurement disagreement be- tween both modalities.
Conclusions: Although TTE is the technique of choice in the follow-up of patients with BAVs, aortic root diam- eter measurements may be inaccurate in the presence of root asymmetry and in BAV with fusion of the right coronary and noncoronary cusps. In these cases, cross-sectional imaging, with MRI or computed tomogra- phy, to confirm aortic diameters may be advisable
Background: In patients with bicuspid valve (BAV), ascending aorta (AAo) dilatation may be caused by altered flow patterns and wall shear stress (WSS). These differences may explain different aortic dilatation morphotypes. Using 4D- flow cardiovascular magnetic resonance (CMR), we aimed to analyze differences in flow patterns and regional axial and circumferential WSS maps between BAV phenotypes and their correlation with ascending aorta dilatation morphotype. Methods: One hundred and one BAV patients (aortic diameter ≤ 45 mm, no severe valvular disease) and 20 healthy subjects were studied by 4D-flow CMR. Peak velocity, flow jet angle, flow displacement, in-plane rotational flow (IRF) and systolic flow reversal ratio (SFRR) were assessed at different levels of the AAo. Peak-systolic axial and circumferential regional WSS maps were also estimated. Unadjusted and multivariable adjusted linear regression analyses were used to identify independent correlates of aortic root or ascending dilatation. Age, sex, valve morphotype, body surface area, flow derived variables and WSS components were included in the multivariable models. Results: The AAo was non-dilated in 24 BAV patients and dilated in 77 (root morphotype in 11 and ascending in 66). BAV phenotype was right-left (RL-) in 78 patients and right-non-coronary (RN-) in 23. Both BAV phenotypes presented different outflow jet direction and velocity profiles that matched the location of maximum systolic axial WSS. RL-BAV velocity profiles and maximum axial WSS were homogeneously distributed right-anteriorly, however, RN-BAV showed higher variable profiles with a main proximal-posterior distribution shifting anteriorly at mid-distal AAo. Compared to controls, BAV patients presented similar WSS magnitude at proximal, mid and distal AAo (p = 0.764, 0.516 and 0.053, respectively) but lower axial and higher circumferential WSS components (p < 0.001 for both, at all aortic levels). Among BAV patients, RN-BAV presented higher IRF at all levels (p = 0.024 proximal, 0.046 mid and 0.002 distal AAo) and higher circumferential WSS at mid and distal AAo (p = 0.038 and 0.046, respectively) than RL-BAV. However, axial WSS was higher in RL-BAV compared to RN-BAV at proximal and mid AAo (p = 0.046, 0.019, respectively). Displacement and axial WSS were independently associated with the root-morphotype, and circumferential WSS and SFRR with the ascending-morphotype
Conclusions: Different BAV-phenotypes present different flow patternswithananteriordistributioninRL-BAV, whereas, RN-BAV patients present a predominant posterior outflow jet at the sinotubular junction that shifts to anterior or right anterior in mid and distal AAo. Thus, RL-BAV patients present a higher axial WSS at the aortic root while RN-BAV present a higher circumferential WSS in mid and distal AAo. These results may explain different AAo dilatation morphotypes in the BAV population
BACKGROUND Imaging biomarkers as predictors of BAV, MFS, and degenerative AAo aneurysms in TAV patients (DA-TAV) are lacking. Biomechanical characterization has been proposed as a possible tool for further aneurysm stratification.
METHODS Two hundred thirty-four subjects (136 BAV, 44 MFS, and 18 DA-TAV patients and 36 healthy control subjects) were included. The cardiac magnetic resonance protocol comprised 4-dimensional flow to assess AAo and descending aorta (DAo) pulse wave velocities (PWVs) and double-oblique, 2-dimensional, steady-state free-precession cine cardiac magnetic resonance to compute aortic distensibility (AD).
RESULTS On adjusted analysis, nondilated BAV patients had similar PWV and AD as healthy control subjects in both AAo and DAo, whereas dilated BAV did not differ from DA-TAV. In contrast, AAo and DAo stiffness in MFS patients was markedly greater than in BAV patients, increasing slightly with dilation severity. AAo PWV showed a biphasic pattern in BAV patients: it first decreased and then increased throughout AAo dilation, with a clear turning point at 50 mm, whereas distensibility did not discern mildly dilated aorta. In multivariate analysis adjusted for clinical and demographic characteristics, only PWV was related to AAo dilation in BAV patients.
CONCLUSIONS The mechanical properties of AAo aneurysms are similar in BAV and TAV patients, whereas MFS patients have a stiffer aorta. Aortic stiffness strongly depends on dilation severity. AAo PWV resulted in a potentially clinically useful biphasic trend with respect to aneurysm diameter, whereas distensibility did not discern mildly dilated aorta. Beyond clinical risk factors, PWV but not AD was independently related to AAo dilation in BAV patients
Aortic dilation in bicuspid aortic valve (BAV) might extend to the proximal arch. Arch flow dynamics and their re- lationship with this segment dilation are still unexplored. Using 4D-flow cardiovascular magnetic resonance, we analysed flow dynamics in the arch for each BAV morphotype and their association with this segment dilation.
Methods
One hundred and eleven BAV patients (aortic diameters <_55mm, non-severe valvular disease), 21 age-matched tri-
and results
cuspid aortic valve (TAV) patients with dilated arch and 24 healthy volunteers (HV) underwent 4D-flow. BAV were classified per fusion morphotype: 75% right-left (RL-BAV), and per arch dilation: 57% dilated, mainly affecting the right-noncoronary (RN) BAV (86% dilated vs. 47% in RL-BAV). Peak velocity, jet angle, normalized displacement, in- plane rotational flow (IRF), wall shear stress, and systolic flow reversal ratio (SFRR) were calculated along the thor- acic aorta. ANCOVA and multivariate linear regression analyses were used to identify correlates of arch dilation. BAV had higher rotational flow and eccentricity than TAV in the proximal arch. Dilated compared with non-dilated BAV had higher IRF being more pronounced in the RN-morphotype. RN-BAV, IRF, and SFRR were independently associated with arch dilation. Aortic stenosis and male sex were independently associated with arch dilation in RL- BAV. Flow parameters associated with dilation converged to the values found in HV in the distal arch.
Conclusions
Increased rotational flow could explain dilation of the proximal arch in RN-BAV and in RL-BAV patients of male sex and with valvular stenosis. These patients may benefit from a closer follow-up with cardiac magnetic resonance or computed tomography