The circle method for preoperative TAVI sizing in a Sievers type 0 stenotic bicuspid aortic valve

2021 ◽  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Circle Method ◽  
Ascending Aorta ◽  
Cardiac Anomaly ◽  
Aortic Valves ◽  
Congenital Cardiac Anomaly ◽  
Video Tutorial ◽  
Transcatheter Heart Valve ◽  
Valve Annulus

The most common congenital cardiac anomaly, affecting an estimated 0.4–2.25% of the general population, is the bicuspid aortic valve. The “pure” bicuspid aortic valve (non-raphe-type or bicuspid aortic valve type 0) is composed of 2 cusps, morphologically and functionally. The shape of the bicuspid aortic valve annulus is often elliptical, is relatively larger than the tricuspid aortic valves, and probably shows severe eccentric calcification. This situation contributes to the difficulties in selecting the correct type and size of transcatheter heart valve when treating bicuspid aortic valve stenosis. Furthermore, it is often associated with a dilated, horizontal ascending aorta and effaced sinuses. The goal of our video tutorial is to present the contemporary circle method used in preoperative sizing during TAVI procedures in patients with a bicuspid aortic valve as well as certain technical considerations and useful advice. Although annular sizing is the main focus for most patients with a bicuspid aortic valve, some patients may need the supra-annular level of sizing. For a dedicated sizing and positioning approach for the SAPIEN 3 Ultra valve, experts in the field propose the circle method.

Fluid-Structure Interaction Predictions of Ascending Aorta Hemodynamics Under Tricuspid and Bicuspid Aortic Valve Flows

2013 ◽  
Author(s):  
Kai Cao ◽  
Philippe Sucosky
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Elastic Fiber ◽  
Ascending Aorta ◽  
Cardiac Anomaly ◽  
Type I ◽  
Matrix Remodeling ◽  
Structural Wall ◽  
Congenital Cardiac Anomaly ◽  
Right Coronary Cusp

The bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly and is present in 2–3% of the general population. As compared to the normal tricuspid aortic valve (TAV) which consists of three leaflets, the most prevalent type-I BAV morphology forms with two as a result of left-/right-coronary cusp fusion. While the BAV anatomy may not intrinsically hamper valvular function, it is associated with a spectrum of secondary aortopathy such as aortic dilation and subsequent dissection. The dilation and thinning of the ascending aorta downstream of a BAV is marked by structural wall abnormalities including smooth muscle cell depletion, elastic fiber degeneration and abnormal extracellular matrix remodeling, which localize to the convexity of the aortic wall.

scholarly journals Bicuspid Aortic Valve Disease and Ascending Aortic Aneurysms: Gaps in Knowledge

2012 ◽  
Vol 2012 ◽  
pp. 1-16 ◽  
Author(s):  
Katie L. Losenno ◽  
Robert L. Goodman ◽  
Michael W. A. Chu
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Disease ◽  
Aortic Aneurysms ◽  
Valve Disease ◽  
Cardiac Anomaly ◽  
Aortic Dilatation ◽  
Congenital Cardiac Anomaly ◽  
Valvular Stenosis ◽  
Ongoing Surveillance

The bicuspid aortic valve is the most common congenital cardiac anomaly in developed nations. The abnormal bicuspid morphology of the aortic valve results in valvular dysfunction and subsequent hemodynamic derangements. However, the clinical presentation of bicuspid aortic valve disease remains quite heterogeneous with patients presenting from infancy to late adulthood with variable degrees of valvular stenosis and insufficiency and associated abnormalities including aortic coarctation, hypoplastic left heart structures, and ascending aortic dilatation. Emerging evidence suggests that the heterogeneous presentation of bicuspid aortic valve phenotypes may be a more complex matter related to congenital, genetic, and/or connective tissue abnormalities. Optimal management of patients with BAV disease and associated ascending aortic aneurysms often requires a thoughtful approach, carefully assessing various risk factors of the aortic valve and the aorta and discerning individual indications for ongoing surveillance, medical management, and operative intervention. We review current concepts of anatomic classification, pathophysiology, natural history, and clinical management of bicuspid aortic valve disease with associated ascending aortic aneurysms.

Role of Hemodynamic Shear Stress Abnormalities in the Early Pathogenesis of Bicuspid Aortic Valve Calcification

2013 ◽  
Author(s):  
Ling Sun ◽  
Santanu Chandra ◽  
Philippe Sucosky
Keyword(s):  
Shear Stress ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
The United States ◽  
Cardiac Anomaly ◽  
Type I ◽  
Calcific Aortic Valve Disease ◽  
Congenital Cardiac Anomaly ◽  
Valvular Calcification ◽  
Right Coronary Cusp

With a prevalence of 1.3 million cases in the United States, the bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly and is frequently associated with calcific aortic valve disease (CAVD) [1]. The most prevalent type-I morphology, which results from left-/right-coronary cusp fusion, generates different hemodynamics than a tricuspid aortic valve (TAV). While valvular calcification has been linked to genetic and atherogenic predispositions, hemodynamic abnormalities are increasingly pointed as potential pathogenic contributors [2–3]. In particular, the wall shear stress (WSS) produced by blood flow on the leaflets regulates homeostasis in the TAV. In contrast, WSS alterations cause valve dysfunction and disease [4]. While such observations support the existence of synergies between valvular hemodynamics and biology, the role played by BAV WSS in valvular calcification remains unknown. The objective of this study was to isolate the acute effects of native BAV WSS abnormalities on CAVD pathogenesis.

Computational Comparison Between Normal and Bicuspid Aortic Valve Hemodynamics

2011 ◽  
Author(s):  
Santanu Chandra ◽  
Clara Seaman ◽  
Nalini M. Rajamannan ◽  
Philippe Sucosky
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Large Degree ◽  
Left Ventricular ◽  
Cardiac Anomaly ◽  
Rapid Progression ◽  
Effective Orifice Area ◽  
Calcific Aortic Valve Disease ◽  
Congenital Cardiac Anomaly ◽  
Hemodynamic Stresses

The bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly and is present in 2% of the population. While a normal tricuspid aortic valve (TAV) consists of three leaflets, a BAV is formed with only two as a result of the fusion of two leaflets into a larger one1. This defect is associated with serious complications such as calcific aortic valve disease (CAVD), a condition characterized by the accumulation of calcium on the leaflets which contributes to the obstruction of the left ventricular outflow and progressive heart failure. Although studies have suggested similarities in the pathogenesis of CAVD in the BAV and TAV, the calcification of the BAV is more severe and its progression more rapid. Previous studies in our laboratory have evidenced the sensitivity of valve leaflets to their hemodynamic environment and the ability of fluid stress alterations to trigger an inflammatory response on the aortic surface of porcine aortic valve leaflets2. Although a similar mechano-etiology could contribute to the rapid calcification of the BAV, it is not clear how the particular BAV anatomy impacts on its hemodynamic environment and whether the hemodynamic stresses experienced by BAV leaflets differ from those present in TAV leaflets. Therefore, the aim of this study was to characterize BAV hemodynamics and to quantify its degree of abnormality relative to a TAV. A fluid-structure interaction (FSI) approach validated with respect to particle-image velocimetry (PIV) measurements was implemented to quantify TAV and BAV hemodynamics in terms of flow velocity field, valvular effective orifice area (EOA) and leaflet wall-shear stress. The large degree of hemodynamic abnormality predicted in the BAV model may contribute to the rapid progression of CAVD in that anatomy. This work lays the foundation for future mechanobiological studies aimed at investigating the isolated effects of native BAV hemodynamic stresses on the development of CAVD.

Abstract 3189: Prevalence of Bicuspid Aortic Valve in Turner Syndrome

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Vandana Sachdev ◽  
Lea Ann Matura ◽  
Stanislav Sidenko ◽  
Vincent Ho ◽  
Andrew Arai ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Turner Syndrome ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Ascending Aorta ◽  
Aortic Dilatation ◽  
Aortic Valves ◽  
Number Of Patients ◽  
Valve Structure ◽  
Asymptomatic Individuals

Women with Turner syndrome (TS) have an increased risk of congenital cardiovascular defects. Previous studies have reported a 10 –20% prevalence rate of bicuspid aortic valves and there are increasing reports of a vasculopathy that predisposes patients to aortic dilatation and dissection. This prospective study aimed to characterize aortic valve and aortic root structure in unselected asymptomatic individuals with TS. A total of 253 females aged 7– 67 years with karyotype proven TS were examined. Transthoracic echocardiography revealed a normal tricuspid aortic valve (TAV) in 162, a ‘probable TAV’ in 8 subjects, a bicuspid aortic valve (BAV) in 65 and ‘probable BAV’ in 3 subjects. The aortic valve could not be visualized by echocardiography in 15/253 or 6%. Magnetic resonance imaging (MRI) revealed valve structure in 11/12 of the probable cases (all confirmatory of the ‘probable’ diagnosis) and 12/15 of the non-visualized cases (8 BAV and 4 TAV), so only 3/253 subjects could not be visualized by either modality. The aortic valve was bicuspid in 76 of the 250 adequately imaged subjects (30%). Peak aortic valve flow was higher in BAV subjects (1.72±0.07 vs. 1.90v0.03 m/sec, P=0.0002), with one case of significant aortic stenosis. Among subjects with a BAV, aortic regurgitation was moderate or greater in ∼15%. Aortic diameters at the annulus, sinuses of Valsalva, sinotubular junction and ascending aorta were all significantly greater in the BAV group. Thirty patients in the BAV group (12%) had aortic root diameters that were outside of the 95% normal confidence limits based on Roman nomograms. Ascending aortic diameters by echo and MRI were highly correlated (r=0.77). In summary, echocardiography supplemented with MRI reveals an extraordinarily high prevalence of abnormal aortic valves in asymptomatic subjects with TS. The abnormal valve structure is associated with higher peak flows, evidence of clinically significant valvular dysfunction, and widening of the ascending aorta in a significant number of patients. All girls and women with TS should have careful echocardiographic evaluation upon diagnosis to identify the one in three asymptomatic individuals with an abnormal valve requiring monitoring for aortic root dilatation and valvular dysfunction.

scholarly journals Developmental changes in bicuspid aortic valve and associated aortopathy in small children

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
J Boesgaard Norsk ◽  
A S Sillesen ◽  
A Axelsson Raja ◽  
M Munk Paerregaard ◽  
C Pihl ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Flow Velocity ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Early Years ◽  
Z Score ◽  
Valve Regurgitation ◽  
Aortic Valve Regurgitation ◽  
Valve Annulus

Abstract Background The prevalence of bicuspid aortic valve (BAV) in newborns is 0.8%. BAV is associated with an increased risk of aortic valve dysfunction and aortopathy. Aortopathy with increased aortic diameters has recently been reported in newborns with BAV. As most patients with BAV are diagnosed in adulthood the development of BAV and associated aortopathy during early years of life is not well described. Purpose The purpose of the study was to assess changes in aortic valve function and aortic dimensions at two to four years of age in children diagnosed with BAV neonatally. Methods Children with BAV were included from a population-based cohort study, in which newborns (n≈25,000) underwent standardized transthoracic echocardiography (TTE). Follow-up TTE was performed and analyzed according to established guidelines. Neonatal (baseline) and follow-up data were compared. Diameters were indexed to body surface area (BSA). Z-scores were calculated using formulas from the Pediatric Heart Network Echocardiogram Database. Results At follow-up 101 newborns with BAV (mean age 2 years 5 months, SD 11 months), 75% male) were examined from May 2019 to April 2021. From baseline to follow-up there was an increase in the BSA-adjusted diameter of the aortic valve annulus (z-score −0.19 at baseline vs 0.95 at follow up, p<0.001), and of the sino-tubular junction (z-score 0.16 at baseline vs 0.43 at follow up, p<0.05). Conversely, there was a decrease in the Z-score for the diameter measured 1 cm from the valve annulus (z-score 1.31 at baseline vs 0.68 at follow up, p<0.001) and at the widest point of the visualized ascending aorta (z-score 2.44 at baseline vs 1.45 at follow up, p<0.001). There was no significant change in the diameter of the sinus of Valsalva (z-score 0.83 at baseline vs 0.80 at follow up, p=0.92). The number of children with at least one aortic z-score >3 was 31 (30.6%) at baseline and 17 (17.8%) at follow up. At baseline mild aortic valve regurgitation was observed in 18 children (17.8%) and in 23 children at follow up (23%). Mean maximum systolic velocities across the aortic valve were 1.03 m/s at baseline (SD 0.24) and 1.11 m/s at follow up (SD 0.27), p<0.05. Aortic stenosis, defined as flow velocity >2.5 m/s was seen in one child both at baseline and at follow-up. Conclusion In children diagnosed with BAV neonatally, re-examination at the age of 2.5 years showed significant increases in the diameter of the aortic valve annulus and the sino-tubular junction, but a significant decrease in the diameter of the ascending aorta. The maximum blood flow velocity across the aortic valve increased and more children had developed mild aortic valve regurgitation. Thus, the bicuspid aortic valve and the associated aortopathy seem to undergo remodeling during early childhood. FUNDunding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The Danish Children's Heart Foundation, Boernehjertefonden.

scholarly journals Bicuspid aortic valve annulus: assessment of geometry and size changes during the cardiac cycle as measured with a standardized method to define the annular plane

2021 ◽  
Author(s):  
Sara Boccalini ◽  
Lidia R. Bons ◽  
Allard T. van den Hoven ◽  
Annemien E. van den Bosch ◽  
Gabriel P. Krestin ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Cardiac Cycle ◽  
Published Data ◽  
Aortic Valves ◽  
Minimum Diameter ◽  
Cardiac Cta ◽  
Bicuspid Aortic Valves ◽  
Valve Annulus ◽  
Standardized Method

Abstract Purpose Bicuspid aortic valve (BAV) is a complex malformation affecting not merely the aortic valve. However, little is known regarding the dynamic physiology of the aortic annulus in these patients and whether it is similar to tricuspid aortic valves (TAV). Determining the BAV annular plane is more challenging than for TAV. Our aim was to present a standardized methodology to determine BAV annulus and investigate its changes in shape and dimensions during the cardiac cycle. Methods BAV patients were prospectively included and underwent an ECG-gated cardiac CTA. The annulus plane was manually identified on reconstructions at 5% intervals of the cardiac cycle with a new standardized method for different BAV types. Based on semi-automatically defined contours, maximum and minimum diameter, area, area-derived diameter, perimeter, asymmetry ratio (AR), and relative area were calculated. Differences of dynamic annular parameters were assessed also per BAV type. Results Of the 55 patients included (38.4 ± 13.3 years; 58% males), 38 had BAV Sievers type 1, 10 type 0, and 7 type 2. The minimum diameter, perimeter, area, and area-derived diameter were significantly higher in systole than in diastole with a relative change of 13.7%, 4.8%, 13.7%, and 7.2% respectively (all p < 0.001). The AR was ≥ 1.1 in all phases, indicating an elliptic shape, with more pronounced flattening in diastole (p < 0.001). Different BAV types showed comparable dynamic changes. Conclusions BAV annulus undergo significant changes in shape during the cardiac cycle with a wider area in systole and a more elliptic conformation in diastole regardless of valve type. Key Points • A refined method for the identification of the annulus plane on CT scans of patients with bicuspid aortic valves, tailored for the specific anatomy of each valve type, is proposed. • The annulus of patients with bicuspid aortic valves undergoes significant changes during the cardiac cycle with a wider area and more circular shape in systole regardless of valve type. • As compared to previously published data, the bicuspid aortic valve annulus has physiological dynamics similar to that encountered in tricuspid valves but with overall larger dimensions.

scholarly journals Bicuspid aortic valve morphology and aortic valvular outflow jets: an experimental analysis using an MRI-compatible pulsatile flow circulation system

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Kaoru Hattori ◽  
Natsuki Nakama ◽  
Jumpei Takada ◽  
Gohki Nishimura ◽  
Ryo Moriwaki ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Pulsatile Flow ◽  
Aortic Wall ◽  
Ascending Aorta ◽  
Circulation System ◽  
Resonance Imaging ◽  
Left Posterior ◽  
Flow Circulation ◽  
The Right

AbstractThe characteristics of aortic valvular outflow jet affect aortopathy in the bicuspid aortic valve (BAV). This study aimed to elucidate the effects of BAV morphology on the aortic valvular outflow jets. Morphotype-specific valve-devising apparatuses were developed to create aortic valve models. A magnetic resonance imaging-compatible pulsatile flow circulation system was developed to quantify the outflow jet. The eccentricity and circulation values of the peak systolic jet were compared among tricuspid aortic valve (TAV), three asymmetric BAVs, and two symmetric BAVs. The results showed mean aortic flow and leakage did not differ among the five BAVs (six samples, each). Asymmetric BAVs demonstrated the eccentric outflow jets directed to the aortic wall facing the smaller leaflets. In the asymmetric BAV with the smaller leaflet facing the right-anterior, left-posterior, and left-anterior quadrants of the aorta, the outflow jets exclusively impinged on the outer curvature of the ascending aorta, proximal arch, and the supra-valvular aortic wall, respectively. Symmetric BAVs demonstrated mildly eccentric outflow jets that did not impinge on the aortic wall. The circulation values at peak systole increased in asymmetric BAVs. The bicuspid symmetry and the position of smaller leaflet were determinant factors of the characteristics of aortic valvular outflow jet.

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