Clinical implications of the biomechanics of bicuspid aortic valve and bicuspid aortopathy

Hassanabad, Ali Fatehi; King, Melissa A.; Martino, Elena S. Di; Fedak, Paul W.M.; García, Julio

doi:10.3389/fcvm.2022.922353

Cited by 9 publications

(7 citation statements)

References 142 publications

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“…An AoD, mainly at the level of the proximal ascending aorta, has been described in a significant percentage of children with BAV, with progression occurring during the pediatric age [ 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 ]. Aortic growth in BAV patients appears to be significantly faster when compared with tricuspid valve patients [ 16 , 17 , 18 , 19 , 20 , 21 , 22 ].…”

Section: Discussionmentioning

confidence: 99%

Aortic Dilatation in Pediatric Patients with Bicuspid Aortic Valve: How the Choice of Nomograms May Change Prevalence

et al. 2023

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Background: Aortic dilation (AoD) is commonly reported in patients with bicuspid aortic valve (BAV) and has been related to flow abnormalities and genetic predisposition. AoD-related complications are reported to be extremely rare in children. Conversely, an overestimate of AoD related to body size may lead to excess diagnoses and negatively impact quality of life and an active lifestyle. In the present study, we compared the diagnosis performance of the newly introduced Q-score (based on a machine-learning algorithm) versus the traditional Z-score in a large consecutive pediatric cohort with BAV. Materials and methods: Prevalence and progression of AoD were evaluated in 281 pediatric patients ages > 5 and < 18 years at first observation, 249 of whom had isolated BAV and 32 had BAV associated with aortic coarctation (CoA–BAV). An additional group of 24 pediatric patients with isolated CoA was considered. Measurements were made at the level of the aortic annulus, Valsalva sinuses, sinotubular aorta, and proximal ascending aorta. Both Z-scores using traditional nomograms and the new Q-score were calculated at baseline and at followup (mean 4.5 years). Results: A dilation of the proximal ascending aorta was suggested by traditional nomograms (Z-score > 2) in 31.2% of patients with isolated BAV and 18.5% with CoA–BAV at baseline and in 40.7% and 33.3%, respectively, at followup. No significant dilation was found in patients with isolated CoA. Using the new Q-score calculator, ascending aorta dilation was detected in 15.4% of patients with BAV and 18.5% with CoA–BAV at baseline and in 15.8% and 3.7%, respectively, at followup. AoD was significantly related to the presence and degree of aortic stenosis (AS) but not to aortic regurgitation (AR). No AoD-related complications occurred during the followup. Conclusions: Our data confirm the presence of ascending aorta dilation in a consistent subgroup of pediatric patients with isolated BAV, with progression during followup, while AoD was less common when CoA was associated with BAV. A positive correlation was found with the prevalence and degree of AS, but not with AR. Finally, the nomograms used may significantly influence the prevalence of AoD, especially in children, with a possible overestimation by traditional nomograms. This concept requires prospective validation in long-term followup.

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Section: Discussionmentioning

confidence: 99%

Aortic Dilatation in Pediatric Patients with Bicuspid Aortic Valve: How the Choice of Nomograms May Change Prevalence

et al. 2023

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“…Alternatively, the hemodynamic theory of bicuspid aortopathy posits that abnormal aortic valve morphology alters laminar blood flow, disrupting vascular wall growth, and function. Recent advances in noninvasive imaging utilize aortic strain, stiffness, and WSS to describe aortic hemodynamics and predict progressive aortic dilation [19 ▪ ,20,21 ▪ –23 ▪ ,40] (see ‘Diagnostic imaging’).…”

Section: Pathologic Mechanisms As Future Prognostic Markersmentioning

confidence: 99%

Bicuspid aortic valve and its ascending aortopathy

Dunne

Lacro

Flyer

2023

Current Opinion in Pediatrics

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Purpose of review To synthesize and critically assess recent clinical and research advancements in pediatric bicuspid aortic valve (BAV) and its associated aortopathy. Recent findings In pediatric patients with BAV, progressive aortic dilation (i.e. bicuspid aortopathy) is commonly present and associated with increased risk for aortic aneurysm, dissection, and surgery in adulthood. Ongoing research explores the cause, incidence, and progression of bicuspid aortopathy to promote earlier diagnosis and improve preventive management. Recent findings include: high familial incidence and need for improved familial screening; safety of recreational physical activity in most affected children; potential for medical management to slow aortic growth; feasibility of pediatric registries to evaluate longitudinal outcomes; and potential genetic and hemodynamic biomarkers for disease risk stratification. Summary Pediatric bicuspid aortopathy is an important area for investigation and preventive management to improve long-term cardiovascular outcomes. Recent literature promotes familial screening, recreational exercise, medical prophylaxis, registry-based longitudinal evaluation, and continued scientific inquiry.

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“…Although valvular complications such as aortic stenosis, aortic regurgitation, and endocarditis are the most common complications, it is well recognized that dilatation and/or aneurysms of the aortic root, ascending aorta and arch -termed bicuspid aortopathy -is observed in up to 50% of individuals with a BAV [1][2][3][4][5][6][7][8][9][10][11][12][13]. Why some patients with BAV develop valvular complications while others demonstrate aortic or mixed presentations remains unclear.…”

Section: Epidemiology Of Bicuspid Aortopathymentioning

confidence: 99%

“…Whether BAV aortopathy results from a genetic defect in the vessel wall, or is a result of altered haemodynamics/rheology (or both) has been a topic of much discussion and debate [2,3,[5][6][7][8][9][10][11][12][13][18][19][20][21][22][23][24][25][26][27][28][29][30][31]. Most BAV are believed to occur sporadically, however autosomal dominant (with variable penetrance), Xlinked, and familial modes of inheritance have also been suggested.…”

Section: Pathophysiology and Clinical Phenotypesmentioning

confidence: 99%

“…Over the past few years, there has been a wealth of data suggesting that altered haemodynamics and valve related rheological changes may be an important basis of BAV aortopathy [3,4,7,[21][22][23][24][25][26][27]29,[33][34][35][36][37][38][39]. Indeed, even a normal functioning BAV valve without stenosis or insufficiency is associated with changes in flow patterns across the valve.…”

Section: Andandmentioning

confidence: 99%

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Bicuspid aortic valve associated aortopathy: 2022 guideline update

Verma

Cohen

Colbert

et al. 2022

Current Opinion in Cardiology

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Purpose of reviewBicuspid aortic valve (BAV) disease is observed in 1--2% of the general population. In addition to valverelated complications (such as aortic stenosis and aortic regurgitation), individuals with BAV often develop dilatation of the proximal aorta (aortic root and ascending aorta), a condition termed BAV aortopathy. The development of BAV aortopathy can occur independent of valvular alterations and can lead to aneurysm formation, aortic dissection or aortic rupture. This review aims to update the clinician with an approach to BAV aortopathy decision making in keeping with the 2022 American College of Cardiology (ACC)/ American Heart Association (AHA) Guideline recommendations. Recent findingsThe ACC/AHA 2022 guidelines provide a contemporary and comprehensive approach to the diagnosis and treatment of aortic pathologies. We review the thresholds for replacement of the aortic root and/or ascending aorta along with the strength and level of evidence recommendations. We also review the various Class 2A and 2B recommendations for earlier intervention, which emphasize the importance of experienced surgeons, and multidisciplinary aortic teams (MATs). SummaryBAV aortopathy is a common and heterogenous clinical problem. The decision making around timing of intervention requires a personalized approach that is based on the aortic dimensions, valve function, rate of growth, family history, patient factors, and surgical experience within MATs.

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Clinical implications of the biomechanics of bicuspid aortic valve and bicuspid aortopathy

Cited by 9 publications

References 142 publications

Aortic Dilatation in Pediatric Patients with Bicuspid Aortic Valve: How the Choice of Nomograms May Change Prevalence

Aortic Dilatation in Pediatric Patients with Bicuspid Aortic Valve: How the Choice of Nomograms May Change Prevalence

Bicuspid aortic valve and its ascending aortopathy

Bicuspid aortic valve associated aortopathy: 2022 guideline update

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