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Enlightening the Association between Bicuspid Aortic Valve and Aortopathy.

ABSTRACT: Bicuspid aortic valve (BAV) patients have an increased incidence of developing aortic dilation. Despite its importance, the pathogenesis of aortopathy in BAV is still largely undetermined. Nowadays, intense focus falls both on BAV morphology and progression of valvular dysfunction and on the development of aortic dilation. However, less is known about the relationship between aortic valve morphology and aortic dilation. A better understanding of the molecular pathways involved in the homeostasis of the aortic wall, including the extracellular matrix, the plasticity of the vascular smooth cells, TGFβ signaling, and epigenetic dysregulation, is key to enlighten the mechanisms underpinning BAV-aortopathy development and progression. To date, there are two main theories on this subject, i.e., the genetic and the hemodynamic theory, with an ongoing debate over the pathogenesis of BAV-aortopathy. Furthermore, the lack of early detection biomarkers leads to challenges in the management of patients affected by BAV-aortopathy. Here, we critically review the current knowledge on the driving mechanisms of BAV-aortopathy together with the current clinical management and lack of available biomarkers allowing for early detection and better treatment optimization.

SUBMITTER: Sophocleous F

PROVIDER: S-EPMC6023468 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature

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Enlightening the Association between Bicuspid Aortic Valve and Aortopathy.

Sophocleous Froso F Milano Elena Giulia EG Pontecorboli Giulia G Chivasso Pierpaolo P Caputo Massimo M Rajakaruna Cha C Bucciarelli-Ducci Chiara C Emanueli Costanza C Biglino Giovanni G

Journal of cardiovascular development and disease 20180419 2

Bicuspid aortic valve (BAV) patients have an increased incidence of developing aortic dilation. Despite its importance, the pathogenesis of aortopathy in BAV is still largely undetermined. Nowadays, intense focus falls both on BAV morphology and progression of valvular dysfunction and on the development of aortic dilation. However, less is known about the relationship between aortic valve morphology and aortic dilation. A better understanding of the molecular pathways involved in the homeostasis ...[more]

PMID: 29671812

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Project description:BackgroundAortic dissection is a life-threatening complication of bicuspid aortic valve (BAV)-associated aortopathy. In these populations, whilst prophylactic replacement of proximal thoracic aortic aneurysms (TAAs) is generally recommended at threshold diameters ≥5.5 cm, dissection may occur in smaller aortas. An alternative size-based parameter, the cross-sectional aortic area/patient height ratio (indexed aortic area, IAA), correlates with increased dissection risk at abnormal values > 10 cm2/m. We sought to assess the utility of the IAA in identifying at-risk BAV-associated TAAs with abnormal IAA, albeit with sub-threshold aortic diameter.Materials and methodsWe retrospectively identified 69 patients with BAV-associated TAAs who underwent surgical repair between 2010 and 2016. Aortic diameter was measured on pre-operative imaging, and IAA calculated, at the mid-sinus of Valsalva, sino-tubular junction and mid-ascending aorta for each patient. We determined proportions of aneurysms with IAA >10 cm2/m, median IAAs corresponding to aortic diameters <4.0 cm, 4.0-4.5 cm, 4.5-5.0 cm, 5.0-5.5 cm and >5.5 cm, and median aortic diameters corresponding to an abnormal IAA.Results50.9%, 12.5% and 64.6% of aneurysms at the sinus of Valsalva, sino-tubular junction and mid-ascending aorta, respectively, had an abnormal IAA. 51.9% and 88.9% of patients with aortic diameter 4.5-5.0 cm and 5.0-5.5 cm, respectively, had an abnormal IAA. In aneurysms with abnormal IAA involving the sinus of Valsalva, sino-tubular junction, and mid-ascending aorta, median aortic diameters were 4.98 cm, 5.04 cm and 5.11 cm, respectively. Overall, 57/72 (79.2%) at-risk aneurysms with IAA >10 cm2/m had diameters smaller than the 5.5 cm guideline cut-off for surgical intervention.ConclusionSignificant proportions of BAV-associated TAAs are at increased risk of aortic dissection attending an IAA >10 cm2/m, whilst not fulfilling the size criteria indicating aortic surgery in contemporary guidelines. Further analysis of IAA in larger BAV cohorts is necessary to clarify its role in patient selection and optimal timing for prophylactic aortic replacement.

Project description:ImportanceThe prevalence and characteristics of bicuspid aortic valve (BAV) are mainly reported from selected cohorts. BAV is associated with aortopathy, but it is unclear if it represents a fetal developmental defect or is secondary to abnormal valve dynamics.ObjectiveTo determine the prevalence of BAV and BAV subtypes and to describe the associated aortopathy in a large, population-based cohort of newborns.Design, setting, and participantsThe Copenhagen Baby Heart Study was a cross-sectional, population-based study open to all newborns born in Copenhagen between April 1, 2016, and October 31, 2018. Newborns with BAV were matched 1:2 to newborns with a tricuspid aortic valve (non-BAV group) on sex, singleton/twin pregnancy, gestational age, weight, and age at time of examination.ExposuresTransthoracic echocardiography within 60 days after birth.Main outcomes and measuresPrimary outcome was BAV prevalence and types, ie, number of raphes and spatial orientation of raphes or cusps (no raphes), according to the classification system of Sievers and Schmidtke (classified as type 0, 1, or 2, with numbers indicating the number of raphes). Secondary outcome was valve function and BAV-associated aortopathy, defined as aortic diameter z score of 3 or greater or coarctation.ResultsIn total, 25 556 newborns (51.7% male; mean age, 12 [SD, 8] days) underwent echocardiography. BAV was diagnosed in 196 newborns (prevalence, 0.77% [95% CI, 0.67%-0.88%]), with male-female ratio 2.1:1. BAV was classified as type 0 in 17 newborns (8.7% [95% CI, 5.5%-13.5%]), type 1 in 178 (90.8% [95% CI, 86.0%-94.1%]) (147 [75.0% {95% CI, 68.5%-80.5%}] right-left coronary raphe, 27 [13.8% {95% CI, 9.6%-19.3%}] right coronary-noncoronary raphe, 4 [2.0% {95% CI, 0.8%-5.1%}] left coronary-noncoronary raphe), and type 2 in 1 (0.5% [95% CI, 0.1%-2.8%]). Aortic regurgitation was more prevalent in newborns with BAV (n = 29 [14.7%]) than in those without BAV (1.3%) (absolute % difference, 13.4% [95% CI, 7.8%-18.9%]; P < .001). Newborns with BAV had higher flow velocities across the valve (0.67 [95% CI, 0.65-0.69] m/s vs 0.61 [95% CI, 0.60-0.62] m/s; mean difference, 0.06 m/s [95% CI, 0-0.1]) and larger aortic root and tubular ascending aortic diameters than those without BAV (10.7 [95% CI, 10.7-10.9] mm vs 10.3 [95% CI, 10.2-10.4] mm; mean difference, 0.43 mm [95% CI, 0.2-0.6 mm] and 9.8 [95% CI, 9.6-10.0] mm vs 9.4 [95% CI, 9.3-9.5] mm; mean difference, 0.46 mm [95% CI, 0.30-0.70], respectively) (P < .001 for all). Aortopathy was seen in 65 newborns (33.2%) with BAV (62 with aortic z score ≥3; 3 with coarctation).Conclusions and relevanceAmong newborns in Copenhagen, the prevalence of BAV was 0.77%. Aortopathy was common in newborns with BAV, suggesting that it also represents a fetal malformation.

Project description:OBJECTIVES:The objectives of this study were to investigate an association between the magnitude of flow-mediated aortic wall shear stress (WSS) and medial wall histopathology in patients with bicuspid aortic valve (BAV) with aortopathy. METHODS:Patients with BAV (n = 27; 52 ± 15 years; 3 women; proximal thoracic aorta diameter = 4.4 ± 0.7 and 4.6 ± 0.5 cm) who underwent prophylactic aortic resection received preoperative 3-dimensional time-resolved phase-contrast magnetic resonance imaging with 3-dimensional velocity encoding to quantify WSS relative to a population of healthy age- and sex-matched tricuspid aortic valve control participants (n = 20). Quantitative histopathology was conducted on BAV aorta tissue samples resected at surgery (n = 93), and correlation was performed between elastic fiber thickness and in vivo aortic WSS as continuous variables. Validation of elastic fiber thickness was achieved by correlation relative to tissue stiffness determined using biaxial biomechanical testing (n = 22 samples). RESULTS:Elastic fibers were thinner and WSS was higher along the greater curvature compared with other circumferential regions (vs anterior wall: P = .003 and P = .0001, respectively; lesser curvature: both P = .001). Increased regional WSS was associated with decreased elastic fiber thickness (r = -0.25; P = .02). Patient stratification with subanalysis showed an increase in the correlation between WSS and histopathology with aortic valve stenosis (r = -0.36; P = .002) and smaller aortic diameters (<4.5 cm: r = -0.39; P = .03). Elastic fiber thinning was associated with circumferential stiffness (r = -0.41; P = .06). CONCLUSIONS:For patients with BAV, increased aortic valve-mediated WSS is significantly associated with elastic fiber thinning, particularly with aortic valve stenosis and in earlier stages of aortopathy. Elastic fiber thinning correlates with impaired tissue biomechanics. These novel findings further implicate valve-mediated hemodynamics in the progression of BAV aortopathy.

Dataset Information

Enlightening the Association between Bicuspid Aortic Valve and Aortopathy.

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Enlightening the Association between Bicuspid Aortic Valve and Aortopathy.

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