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Valvular aortic stenosis with a bicuspid valve. A full volume rendered three-dimensional echocardiographic "surgeon's view" of a bicuspid aortic valve in systole showing fused right (RCC) and noncoronary (NCC) cusps with prominent ridge, a smaller left coronary cusp (LCC) (lower to the orifice) and an eccentric elliptical restricted orifice (in light blue color).
Source publication
Aortic valve stenosis in children is a congenital heart defect that causes fixed form of hemodynamically significant left ventricular outflow tract obstruction with progressive course. Neonates and young infants who have aortic valve stenosis, usually develop congestive heart failure. Children and adolescents who have aortic valve stenosis, are mostly...
Context in source publication
Context 1
... and threedimensional echocardiography demonstrate the valve morphology, aortic root dimensions, the presence of commissural fusion or raphes and the position and orientation of the valve orifice. The "surgeon's view" (Figure 1) by three-dimensional echocardiography delineate the surgical anatomy and anatomic orifice area of the valve that can help decide the type of valve surgery or intervention. Bicuspid aortic valves due to right and left coronary leaflet cusp fusion have less risk whereas those with other types of fusion of leaflets have more risk of the occurrence of VAS and associated aortopathy. ...
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Citations
... Aortic valve (AV) stenosis is a rare congenital cardiac malformation with a prevalence of 2.2-6% [1,2]. "Critical" aortic valve stenosis (CAVS) implies a severe impairment of the left ventricle (LV) due to a high-grade stenosis and pressure overload of the LV. ...
... AV dysplasia and hypoplasia of the aortic annulus are the main causes. In most cases, the AV has a unicuspid or bicuspid morphology [2,4] due to congenital commissural fusions. Additional cardiac malformations may exist, such as aortic hypoplasia or coarctation of the aorta. ...
Congenital critical aortic valve stenosis (CAVS) is a life-threatening disease requiring urgent treatment. First-line therapy is still controversial. The aim of our study was (1) to analyze retrospectively the patients of our institution who underwent balloon aortic valvuloplasty (BAV) due to CAVS and (2) describe the techniques for improved feasibility of intervention using microcatheters and retrieval loops. Twelve patients underwent 23 BAVs: 1 BAV was performed in 3 patients, 2 BAVs were performed in 7 patients, and 3 BAVs were performed in 2 patients. The peak trans-valvular pressure gradient (Δp) and left ventricular shortening fraction (LVSF) improved significantly in the first two interventions. In the first BAV, Δp decreased from 73.7 ± 34.5 mmHg to 39.8 ± 11.9 mmHg (p = 0.003), and the LVSF improved from 22.3 ± 13.5% to 31.6 ± 10.2% (p = 0.001). In the second BAV, Δp decreased from 73.2 ± 33.3 mmHg to 35.0 ± 20.2 mmHg (p < 0.001), and the LVSF increased from 26.7 ± 9.6% to 33.3 ± 7.4% (p = 0.004). Cardiac surgery during the neonatal period was avoided for all children. The median time to valve surgery was 5.75 years. Few complications occurred, namely mild-to-moderate aortic regurgitation, one remediable air embolism, and one intimal injury to the ascending aorta. We conclude that BAV is a successful emergency treatment for CAVS, resulting in left ventricular relief, clinical stabilization, and a time gain until cardiac surgery.
... Congenital aortic valve malformations include aortic atresia (1), aortic stenosis (2,3) and bicuspid aortic valves (BAVs) (4). Among these, BAVs are the most common, affecting more than 2% of the global population (4,5). ...
Mouse models of congenital aortic valve malformations are useful for studying disease pathobiology, but most models have incomplete penetrance (e.g., ~2 to 77% prevalence of bicuspid aortic valves (BAVs) across multiple models). For longitudinal studies of pathologies associated with BAVs and other congenital valve malformations, which manifest over months in mice, it is operationally inefficient, economically burdensome, and ethically challenging to enroll large numbers of mice in studies without first identifying those with valvular abnormalities. To address this need, we established and validated a novel in vivo high frequency (30 MHz) ultrasound imaging protocol capable of detecting aortic valvular malformations in juvenile mice. Fifty natriuretic peptide receptor 2 heterozygous mice on a low density lipoprotein receptor deficient background (Npr2+/-;Ldlr-/-; 32 male, 18 female) were imaged at 4- and 8-weeks of age. Fourteen percent of the Npr2+/-;Ldlr-/- mice exhibited features associated with aortic valve malformations, including: i) abnormal trans-aortic flow patterns on color Doppler (recirculation and regurgitation); ii) peak systolic flow velocities distal to the aortic valves reaching or surpassing ~1250 mm/s by pulsed wave Doppler; and iii) putative fusion of cusps along commissures and abnormal movement elucidated by 2D imaging with ultra-high temporal resolution. Valves with these features were confirmed by ex vivo gross anatomy and histological visualization to have thickened cusps, partial fusions, or Sievers type 0 bicuspid valves. This ultrasound imaging protocol will enable efficient, cost-effective and humane implementation of studies of congenital aortic valvular abnormalities and associated pathologies in a wide range of mouse models.
... Six of the top 10 author keywords were related to the aortic valve, indicating increased focus on the health management of patients with aortic valve replacement. Aortic valve stenosis is the most common VHD (36)(37)(38)(39). The incidence of aortic valve disease is expected to increase due to the prevalence of risk factors, such as high blood pressure and diabetes. ...
Background
Valvular heart disease is a major health concern worldwide. The effective management of patients undergoing valve replacement determines their prognosis. Bibliometric analysis of studies on managing patients with artificial heart valves has not been previously performed.
Methods
This study analyzed 2,771 publications related to patient management after valve replacement published in the Web of Science Core Collection database between January 1, 2013, and December 31, 2022. Bibliometric analysis was performed using CiteSpace and VOSviewer considering countries, institutions, authors, journals, references, and keywords.
Results
The countries with the most significant contributions in this field were the United States of America (USA), Germany, and Italy. Leon MB from Columbia University, USA was the most influential author. Transcatheter aortic valve replacement was a current research hotspot, while anticoagulation management was a key area of interest. Combining anticoagulation therapy with internet-linked tools and portable health devices may offer new research avenues. Frailty assessment and intervention were potential future research areas.
Conclusions
This bibliometric analysis provides clinicians and researchers with useful insights for developing novel ideas and directions to manage the health of patients undergoing valve replacement.
... The aortic valve is located between the left ventricle (LV) and ascending thoracic aorta (6). Like all valves in the heart, it serves to maintain unidirectional flow. ...
... Recently, the probability of requiring balloon valvuloplasty is shown to be 20% in patients with catheter-measured peak pressure gradients less than 25 mmHg, and 40% and 70% in patients with gradients 25-49 mmHg and >50 mmHg, respectively (59). Notably, congenital AVS is a progressive disorder as the risk of morbid events such as heart failure, sudden death, and ventricular arrhythmia increase at a rate of 1%-1.5% per year, if left untreated (6,59). Similarly, the risk of developing AVS in children with isolated BAV increases along with age (6). ...
... Notably, congenital AVS is a progressive disorder as the risk of morbid events such as heart failure, sudden death, and ventricular arrhythmia increase at a rate of 1%-1.5% per year, if left untreated (6,59). Similarly, the risk of developing AVS in children with isolated BAV increases along with age (6). As with all CHD, bacterial endocarditis remains a potential complication of AVS, with an incidence of 27.1 per 10,000 person years (60). ...
Congenital aortic valve stenosis (AVS) is one of the most common valve anomalies and accounts for 3%–6% of cardiac malformations. As congenital AVS is often progressive, many patients, both children and adults, require transcatheter or surgical intervention throughout their lives. While the mechanisms of degenerative aortic valve disease in the adult population are partially described, the pathophysiology of adult AVS is different from congenital AVS in children as epigenetic and environmental risk factors play a significant role in manifestations of aortic valve disease in adults. Despite increased understanding of genetic basis of congenital aortic valve disease such as bicuspid aortic valve, the etiology and underlying mechanisms of congenital AVS in infants and children remain unknown. Herein, we review the pathophysiology of congenitally stenotic aortic valves and their natural history and disease course along with current management strategies. With the rapid expansion of knowledge of genetic origins of congenital heart defects, we also summarize the literature on the genetic contributors to congenital AVS. Further, this increased molecular understanding has led to the expansion of animal models with congenital aortic valve anomalies. Finally, we discuss the potential to develop novel therapeutics for congenital AVS that expand on integration of these molecular and genetic advances.
... After a period of observation, some of them may be stable enough to be discharged and evaluated in a cardiac center in 1-2 weeks with balloon dilation as soon as possible in the first month of life. Careful assessment in collaboration with the accepting center should be considered before discharging such newborn [29]. ...
Critical congenital heart diseases (CCHD) are important causes of mortality and morbidity in the newborn period. Even after diagnosis, their management could be seriously compromised by the unplanned delivery in hospitals with limited expertise and resources. The newborn may spend days or week before transport putting a significant burden on the neonatal team to manage such challenging diseases. In this review, the management principles of each individual pathology are discussed in the setting before transport to cardiac centers. Understanding these principles will help the treating teams evaluate and manage those complex conditions. The review avoids the advanced discussion on the management of CCHDs not applicable to the pre-transport setup. It highlights the critical elements in the maintenance of normal hemodynamics specific to each pathology and their variation.
... Ex vivo analysis shows that abnormal hemodynamic shear stress may contribute to early adults CAVS pathogenesis (67). Additionally, pediatric and adult cases of BAV experience similar hemodynamic alterations (68). In environments with excessive shear stress such as the AV, enzyme-induced turnover of ECM is essential to maintain homeostasis. ...
In heart valve biology, organization of the extracellular matrix structure is directly correlated to valve function. This is especially true in cases of pediatric congenital aortic valve stenosis (pCAVS), in which extracellular matrix (ECM) dysregulation is a hallmark of the disease, eventually leading to left ventricular hypertrophy and heart failure. Therapeutic strategies are limited, especially in pediatric cases in which mechanical and tissue engineered valve replacements may not be a suitable option. By identifying mechanisms of translational and post-translational dysregulation of ECM in CAVS, potential drug targets can be identified, and better bioengineered solutions can be developed. In this review, we summarize current knowledge regarding ECM proteins and their post translational modifications (PTMs) during aortic valve development and disease and contributing factors to ECM dysregulation in CAVS. Additionally, we aim to draw parallels between other fibrotic disease and contributions to ECM post-translational modifications. Finally, we explore the current treatment options in pediatrics and identify how the field of proteomics has advanced in recent years, highlighting novel characterization methods of ECM and PTMs that may be used to identify potential therapeutic strategies relevant to pCAVS.
... These devices can only achieve simple concentric-like constriction of the aorta and fail to re-create the complex three-dimensional (3D) flow patterns observed in AS created by maladaptive geometry from calcification or congenital defects of leaflet valves. Moreover, their limited control prevents them from recapitulating the haemodynamics of congenital aortic valve defects, which often accelerate the onset and progression of AS as well as aortic remodelling, potentially leading to other complications including aortic aneurysms, dissection and regurgitation 24 . In this work, we focus on re-creating the haemodynamics of calcific AS, as well as of bicuspid or bicommissural aortic valve (BAV)-the most common congenital valve disease 11,25,26 -and unicommissural aortic valve (UAV), a rare congenital defect that is typically associated with an even poorer prognosis than BAV [27][28][29] . ...
Preclinical models of aortic stenosis can induce left ventricular pressure
overload and coarsely control the severity of aortic constriction. However,
they do not recapitulate the haemodynamics and flow patterns associated
with the disease. Here we report the development of a customizable soft
robotic aortic sleeve that can mimic the haemodynamics and biomechanics
of aortic stenosis. By allowing for the adjustment of actuation patterns and
blood-flow dynamics, the robotic sleeve recapitulates clinically relevant
haemodynamics in a porcine model of aortic stenosis, as we show via
in vivo echocardiography and catheterization studies, and a combination
of in vitro and computational analyses. Using in vivo and in vitro magnetic
resonance imaging, we also quantified the four-dimensional blood-flow
velocity profiles associated with the disease and with bicommissural and
unicommissural defects re-created by the robotic sleeve. The design of the
sleeve, which can be adjusted on the basis of computed tomography data,
allows for the design of patient-specific devices that may guide clinical
decisions and improve the management and treatment of patients with
aortic stenosis.
... Risk factors such as bicuspid aortic valves (BAV), diabetes, mechanical injury, hypertension, maleness, smoking, and hypercholesterolemia all contribute to the development and progression of this disease (2, 3, 5, 6). It is important to note that AVS accounts for 3-6% of congenital heart defects in neonates and infants, often developing during the first trimester and evolving throughout gestation (7). With the only successful treatment option for AVS being surgical valve replacement, there is an urgent need to develop new target therapies (3). ...
Background
CXCL12/CXCR4 signaling is essential in cardiac development and repair, however, its contribution to aortic valve stenosis (AVS) remains unclear. In this study, we tested the role of endothelial CXCR4 on the development of AVS.
Materials and methods
We generated CXCR4 endothelial cell-specific knockout mice (EC CXCR4 KO) by crossing CXCR4 fl/fl mice with Tie2-Cre mice to study the role of endothelial cell CXCR4 in AVS. CXCR4 fl/fl mice were used as controls. Echocardiography was used to assess the aortic valve and cardiac function. Heart samples containing the aortic valve were stained using Alizarin Red for detection of calcification. Masson’s trichrome staining was used for the detection of fibrosis. The apex of the heart samples was stained with wheat germ agglutinin (WGA) to visualize ventricular hypertrophy.
Results
Compared with the control group, the deletion of CXCR4 in endothelial cells led to significantly increased aortic valve peak velocity and aortic valve peak pressure gradient, with decreased aortic valve area and ejection fraction. EC CXCR4 KO mice also developed cardiac hypertrophy as evidenced by increased diastolic and systolic left ventricle posterior wall thickness (LVPW), cardiac myocyte size, and heart weight (HW) to body weight (BW) ratio. Our data also confirmed increased microcalcifications, interstitial fibrosis, and thickened valvular leaflets of the EC CXCR4 KO mice.
Conclusion
The data collected throughout this study suggest the deletion of CXCR4 in endothelial cells is linked to the development of aortic valve stenosis and left ventricular hypertrophy. The statistically significant parameters measured indicate that endothelial cell CXCR4 plays an important role in aortic valve development and function. We have compiled compelling evidence that EC CXCR4 KO mice can be used as a novel model for AVS.
... While in the bicuspid aortic valve, the balloon dilatation tears the fused commissures with adequate relief of obstruction and some valvular regurgitation, in the unicuspid valve, balloon dilatation tends to split the leaflet opposite the patent commissure with only partial relief of obstruction and significant valvular regurgitation. [1] However, studies have shown different results regarding this association. In a study, compared with other valve morphologies, patients with bicuspid aortic valves experienced diminished freedom from re-intervention, death, or transplant. ...
... In the current study, the re-intervention rate was detected to be 17, 3% and it is associated with immediate outcomes of the procedure. Singh [1] reported that being under 3 months of age and having immediate postprocedural peak gradient >30 mmHg are considered predictors of restenosis. According to a study conducted by Sullivan PM et al. [8] , neonatal age, additional left heart lesions, and preintervention aortic valve gradient were not associated with the risk of aortic valve replacement. ...
... Neonatal ABV mortality in an intermediate-term follow-up has been reported as 9.3% and 12% in other studies. [1,4] There were no intraprocedural deaths in the current study, however, the hospital mortality was 21.2%. We noticed that most of the patients who did not survive (64%) were patients who had an ABV between the years 2007 and 2012 which may be due to the lack of experience and equipment or postprocedural care. ...
Introduction: Critical or severe aortic stenosis in new-borns is a condition that requires rapid intervention. Aortic balloon valvuloplasty (ABV) is a method of choice that has been successfully performed since 1983. Aims: This study was conducted to explore the experiences of our centre. Study Design: The data of ABV performed on new-borns (n = 52) between 2007 and 2020 were retrospectively analysed to evaluate follow-up of the cases. Materials and Methods: Patients were divided into 4 groups according to procedural immediate results. Results: Left ventricular endocardial fibroelastosis and left ventricular systolic dysfunction were detected in 18 (34.6%) and 19 (36.5%) patients, respectively and there was a significant association between fibroelastosis and left ventricular dysfunction (P < 0.05). The preprocedural echocardiographic mean gradient was significantly lower in the unsuccessful group (P < 0.41). The mean hospital stay day was shorter in the group with optimal results without statistical significance (P = 0.055). Immediate inadequate results after the procedure were detected as a major risk for re-intervention. Re-intervention was required in one-fifth of the patients and the most common cause was aortic stenosis. The risk factors of mortality were found to be associated with the disease itself such as ventricular dysfunction, being critical aortic stenosis instead of procedural reasons. Conclusion: ABV is an effective method and as left ventricular dysfunction and critical aortic stenosis are risk factors of mortality, preprocedural evaluation, and quick intervention are essential.
... The transstenotic pressure gradient is proportional to the flow [67,68] and can be used to evaluate the severity of a vascular disease. Pressure gradients are routinely measured noninvasively in the clinic [69,70] and are usually calculated from blood flow velocities obtained using SDUS by applying the simplified Bernoulli equation [66]. This method does not describe the pressure gradient across a constriction properly [71,72], but still remains the most commonly used method on US scanners. ...
... The estimations were accurate and highly precise when compared with computer simulated flow. Intravascular pressure gradients are routinely measured using echocardiography in the pediatric patients [70], but the measurements can be highly variable as discussed in study I. The VFI method in our study achieved a strong correlation for the pressure increases and high accuracy relative to the computer simulated flow. ...
This PhD thesis is a continuation of the collaborative work between engineers
employed at the Technical University of Denmark or BK Medical as well as medical
doctors employed at Rigshospitalet.
Blood velocities are routinely measured in the clinic using ultrasound (US) to
identify vessel pathology. US is the preferred method for measuring blood velocities,
since it is relatively cheap, requires no ionizing radiation, and can be performed
bedside. However, conventional Doppler ultrasound is angle-dependent and
requires manual alignment of the beam and flow angle before accurate velocity
estimation can be achieved. Beam and flow alignment can be particularly
challenging when vessel pathology is present, but this issue can be circumvented
entirely with an US method called Vector Flow Imaging (VFI).
This PhD thesis investigated the VFI method as a tool for evaluating the severity of
constricted dialysis fistulas and heart valves. Additionally, VFI was also used to
estimate pressure gradients in carotid bifurcations. The aim was to evaluate the
clinical applications of VFI to see if the method would be able to contribute to the
clinical daily work.
In study I, correlations between noninvasively measured pressure gradients and
pressure gradients measured with an invasive pressure catheter were evaluated in a
review. Overall, magnetic resonance imaging and Doppler US correlated well with
invasive catheterization.
In study II, VFI was used to measure the pressure gradients between the common
carotid artery and the carotid bulb in seven phantom models. VFI followed the same
7 trends in pressure decrease and increase as the computer simulated models, whereas
the invasive pressure catheter was not sensitive enough to measure the subtle
pressure gradients found in this environment.
In study III, transstenotic turbulences in dysfunctional dialysis fistulas were
visualized with VFI and used to assess the severity of vascular constrictions. VFI
showed a moderate to strong correlation with digital subtraction angiography in five
patients and moderate correlation with spectral Doppler ultrasound peak velocities
in 12 patients. A phantom substudy showed that turbulence estimation with VFI was
a reliable parameter even in aliased flow.
In study IV, transvalvular turbulence was evaluated as a measure of valve stenosis
severity in small children with congenital heart valve stenosis. A strong correlation
was found between VFI and catheterization before treatment. Additionally, a strong
correlation was found between the decrease in pressure gradient measured using a
catheter and decrease in turbulence measured using VFI.
The clinical studies showed an association between VFI-derived flow complexity
and the currently used clinical standards. However, the sample sizes were small and
the possible impact the results may have on clinical routines is, therefore, bland.
We implore more research groups to conduct comparative studies to verify or
disprove our findings. VFI offers more information about the blood flow than
conventional US and may increase diagnostic certainty if used routinely in the clinic.
