An economic analysis of medical and surgical management of aortopathy associated with bicuspid aortic valve.
Hardikar Ashutosh,Marwick Thomas H
European heart journal. Quality of care & clinical outcomes
AIMS:To develop a cost-effectiveness model to address the outcome and economic implications of different thresholds for surgery in the management of aortopathy associated with bicuspid aortic valve disease. METHODS AND RESULTS:A model was created from the perspective of an Australian healthcare funding agency. The index case was a 65-year-old with bicuspid aortic valve (BAV) and ascending aorta diameter of 5.0 cm. Health states were defined as: pre-operative with dilated aorta, post-operative without complications, post-complication, and death. The mean and variance of risks and transition probabilities were taken from a local surgical database and local costs and utilities of elective and urgent thoracic aortic surgery (AoS) with or without aortic valve replacement, with a sensitivity analysis based on a systematic review. Scenario analyses were provided for other aortic dimensions. Implications for survival, quality-adjusted life years (QALYs), and costs were calculated from healthcare delivery and economic perspectives. After 10 000 simulations for the reference case, the utility of watchful waiting (WW) exceeded that of elective AoS (13 ± 4 vs. 10 ± 5 QALY). The net monetary benefit was A$351 063 ± 304 965 with immediate AoS vs. 534 797 ± 198 570 with WW surveillance. The most important variables affecting effectiveness were utility value of survivors, rate of aortic growth, and probability of acute aortic event during WW. CONCLUSIONS:This decision-analytic model informed by our practice, as well as a systematic analysis, shows that AoS in a BAV patient with aorta <5 cm diameter is costlier and less effective than WW.
10.1093/ehjqcco/qcz020
Early Prognosis of Reduction Ascending Aortoplasty in Patients With Aortic Valve Disease: A Single Center's Experience.
Liu Shen,Shi Yi,Liu Ruofan,Tong Minghui,Luo Xiang,Xu Jianping
The Annals of thoracic surgery
BACKGROUND:Dilatation of the ascending aorta tends to develop in individuals with aortic valve disease. Reduction ascending aortoplasty (RAA) is an alternative procedure to ascending aortic replacement. This study was designed to identify the early prognosis of RAA for patients with aortic valve disease and dilatation of the ascending aorta. METHODS:From January 2010 to December 2013, 102 patients with aortic valve disease and ascending aortic dilatation who had undergone aortic valve replacement combined with RAA were enrolled. Fifty-seven patients with bicuspid aortic valve (BAV group) and 45 patients without BAV (non-BAV group) were analyzed. The study endpoints were postoperative death, reoperation, stroke, and aorta-related adverse events. RESULTS:Follow-up data were obtained from all patients. The mean follow-up time was 38.8 ± 13.0 months. More patients with mitral valve disease and tricuspid insufficiency were present in the non-BAV group. More patients with ascending aorta larger than 45 mm were present in the BAV group. Two patients died, and 1 patient experienced a stroke. There were no reoperations or aorta-related adverse events. The mean expansion degree and rate of ascending aorta were 0.39 ± 0.26 cm and 1.3 ± 0.8 mm/year, respectively, in patients with aortic redilatation. Redilatation was observed in the BAV group (37.0 ± 5.0 mm vs 35.5 ± 4.6 mm; p = 0.009), whereas the non-BAV group had no significant change. CONCLUSIONS:Reduction ascending aortoplasty shows good early results in patients with aortic valve disease and dilatation of the ascending aorta. Redilatation tends to happen in patients with BAV, and long-term follow-up is necessary.
10.1016/j.athoracsur.2016.06.005
The impact of the aortic cusps fusion pattern and valve disease severity on the aortic wall mechanics in patients with bicuspid aortic valve.
Kalinowski Mariusz E,Szulik Mariola,Pawlak Szymon,Rybus-Kalinowska Barbara,Zembala Marian,Kalarus Zbigniew,Kukulski Tomasz
The international journal of cardiovascular imaging
The ascending aorta dilatation in the bicuspid aortic valve (BAV) patients is often attributed to congenital abnormalities of the aortic wall, but it may be related to hemodynamic disturbances in the course of BAV disease. At present, ascending aortic diameter is used as almost sole but weak predictor of aortic dissection and rupture in BAV. We examined the association between aortic wall mechanics and severity of aortic valve disease including different cusps fusion patterns using conventional echocardiography and tissue Doppler imaging (TDI). We prospectively studied 106 BAV patients: 72 with right-left (R-L) coronary cusp fusion were matched 1:1 to 34 patients with right-noncoronary (R-N) cusp fusion obtaining 34 pairs of patients. Peak systolic radial velocity and acceleration of the ascending aortic wall, measured by TDI, were used as an index of hemodynamic stress imposed on the aorta. Paired analysis showed higher aortic wall radial velocity (4.71 ± 1.61 cm/s vs. 3.33 ± 1.44 cm/s, p = 0.001) and acceleration (1.08 ± 0.46 m/s vs. 0.80 ± 0.34 m/s, p = 0.015) in-R-L compared to R-N fusion. Pearson correlation showed association of ascending tubular aortic diameter with age (r = 0.258, p = 0.012), weight (r = 0.323, p = 0.001), peak aortic valve gradient (r = 0.386, p = 0.0001), aortic root diameter (r = 0.439, p < 0.0001), and R-N fusion pattern (r = 0.209, p = 0.043). Aortic root diameter was related to male gender (r = 0.296, p = 0.003), weight (r = 0.381, p = 0.0001), ascending aortic diameter (r = 0.439, p < 0.0001), and severity of aortic regurgitation (r = 0.337, p = 0.0009). Regional differences in aortic wall motion between different BAV cusp fusion patterns and association of aortic diameters with the severity of aortic valve disease, both suggest a deleterious hemodynamic impact of cusp fusion patterns and aortic valve dysfunction on ascending aortic wall. Assessment of aortic hemodynamic by TDI is feasible and could be potentially used to improve prediction of acute aortic complications, thus helping to establish optimal timing of aortic surgery in BAV patients.
10.1007/s10554-020-01838-0
Long-term Fate of Dilated Ascending Aorta after Aortic Valve Replacement for Bicuspid Versus Tricuspid Aortic Valve Disease.
Kim Min-Seok,Kim Jung Hwan,Lee Seung Hyun,Lee Sak,Youn Young-Nam,Yoo Kyung-Jong,Joo Hyun-Chel
The American journal of cardiology
We compared the long-term outcomes and difference in dilatation rates of the ascending aorta after aortic valve (AV) replacement (AVR) between bicuspid and tricuspid AV patients, and evaluated risk factors associated with ascending aorta dilatation and aortic events during the follow-up. Of 1,127 patients who underwent AVR from 1995 to 2015, 259 patients with a dilated ascending aorta (≥40 mm in diameter) were included. The patients were divided into those with bicuspid (group bicuspid aortic valve [BAV], n = 105) and with tricuspid (group tricuspid aortic valve [TAV], n = 154) AV, and a propensity score-matched analysis was performed to match 98 patients in each group. The differences in the dilation rate of the ascending aorta and long-term outcomes were analyzed. Risk factors for ascending aorta dilatation, mortality, and aortic events were identified. Follow-up was completed in 100% of patients with a median follow-up duration of 106.1 [68.8, 163.0] months. The early clinical outcomes and dilation rate of the ascending aorta were similar between the groups. Overall survivals up to 15 years postoperatively were similar between groups BAV and TAV (p = 0.223). Aortic events occurred in 6 patients (groups BAV vs TAV, 2 vs 4;p = 0.678). Preoperative ascending aorta diameter showed a linear relationship with the dilatation rate of ascending aorta (p <0.001) and was related to progressive aortic dilatation and aortic events (odds ratio: 1.25, p <0.001 and hazard ratio = 1.56, p <0.001, respectively). In conclusion, the long-term outcomes and ascending aorta dilatation rate were similar between the BAV and TAV patients up to 15 years after AVR. Bicuspid AV was not a risk factor of mortality or aortic events.
10.1016/j.amjcard.2020.05.026
Late post-AVR progression of bicuspid aortopathy: link to hemodynamics.
Naito Shiho,Gross Tatiana,Disha Kushtrim,von Kodolitsch Yskert,Reichenspurner Hermann,Girdauskas Evaldas
General thoracic and cardiovascular surgery
BACKGROUND AND AIM OF THE STUDY:The ascending aortic dilatation may progress after aortic valve replacement (AVR) in bicuspid aortic valve (BAV) patients. Our aim was to evaluate rheological flow patterns and histological characteristics of the aneurysmal aorta in BAV patients at the time of reoperative aortic surgery. MATERIALS AND METHODS:13 patients (mean age: 42 ± 9 years, 10 (77%) male) with significant progression of proximal aortopathy after isolated AVR surgery for BAV disease (i.e., 16.7 ± 8.1 years post-AVR) were identified by cardiac phase-contrast cine magnetic resonance imaging (MRI) in our hospital. A total of nine patients (69%) underwent redo aortic surgery. Based on the MRI data, the aortic area of the maximal flow-induced stress (jet sample) and the opposite site (control sample) were identified and corresponding samples were collected intraoperatively. Histological sum-score values [i.e. aortic wall changes were graded based on a summation of seven histological criteria (each scored from 0 to 3)] were compared between these samples. RESULTS:Mean proximal aortic diameter at MRI follow-up was 55 ± 6 mm (range 47-66mm). Preoperative cardiac MRI demonstrated eccentric systolic flow pattern directed towards right-lateral/right posterior wall of the proximal aorta in 9/13 (69%) patients. Histological sum-score values were significantly higher in the jet sample vs control sample (i.e., 8.3 ± 3.8 vs 5.6 ± 2.4, respectively, p = 0.04). CONCLUSIONS:Hemodynamic factors may still be involved in the late progression of bicuspid aortopathy even after isolated AVR surgery for BAV disease.
10.1007/s11748-017-0746-4
Hemodynamics alteration in patient-specific dilated ascending thoracic aortas with tricuspid and bicuspid aortic valves.
Jayendiran Raja,Campisi Salvatore,Viallon Magalie,Croisille Pierre,Avril Stéphane
Journal of biomechanics
In this paper, we evaluate computationally the influence of blood flow eccentricity and valve phenotype (bicuspid (BAV) and tricuspid (TAV) aortic valve) on hemodynamics in ascending thoracic aortic aneurysm (ATAA) patients. 5 TAV ATAA, 5 BAV ATAA (ascending aorta diameter >35 mm) and 2 healthy subjects underwent 4D flow MRI. The 3D velocity profiles obtained from 4D flow MRI were given as input boundary conditions to a computational fluid dynamics analysis (CFD) model. After performing the CFD analyses, we verified that the obtained time-averaged velocity profiles and flow eccentricity were in good agreement with 4D flow MRI. Then we used the CFD analyses to evaluate the time-averaged wall shear stress (TAWSS) and the local normalized helicity (LNH). We found that the flow eccentricities at the aortic root were not significantly different (p > 0.05) between TAV and BAV phenotypes. TAWSS (R = 0.697, p = 0.025) and absolute LNH (R = 0.964, p < 0.001) are in good correlation with flow eccentricity. We conclude that eccentricity at the aortic root is a major determinant of hemodynamics patterns in ATAA patients regardless of the aortic valve phenotype.
10.1016/j.jbiomech.2020.109954
Patterns of ascending aortic dilatation and predictors of surgical replacement of the aorta: A comparison of bicuspid and tricuspid aortic valve patients over eight years of follow-up.
Agnese Valentina,Pasta Salvatore,Michelena Hector I,Minà Chiara,Romano Giuseppe Maria,Carerj Scipione,Zito Concetta,Maalouf Joseph F,Foley Thomas A,Raffa Giuseppe,Clemenza Francesco,Pilato Michele,Bellavia Diego
Journal of molecular and cellular cardiology
BACKGROUND:Predictors of thoracic aorta growth and early cardiac surgery in patients with bicuspid aortic valve are undefined. Our aim was to identify predictors of ascending aorta dilatation and cardiac surgery in patients with bicuspid aortic valve (BAV). METHODS:Forty-one patients with BAV were compared with 165 patients with tricuspid aortic valve (TAV). All patients had LV EF > 50%, normal LV dimensions, and similar degree of aortic root or ascending aorta dilatation at enrollment. Patients with more than mild aortic stenosis or regurgitation were excluded. A CT-scan was available on 76% of the population, and an echocardiogram was repeated every year for a median time of 4 years (range: 2 to 8 years). Patterns of aortic expansion in BAV and TAV groups were analyzed by a mixed-effects longitudinal linear model. In the time-to-event analysis, the primary end point was elective or emergent surgery for aorta replacement. RESULTS:BAV patients were younger, while the TAV group had greater LV wall thickness, arterial hypertension, and dyslipidemia than BAV patients. Growth rate was 0.46 ± 0.04 mm/year, similar in BAV and TAV groups (p = 0.70). Predictors of cardiac surgery were aorta dimensions at baseline (HR 1.23, p = 0.01), severe aortic regurgitation developed during follow-up (HR 3.49, p 0.04), family history of aortic aneurysm (HR 4.16, p 1.73), and history of STEMI (HR 3.64, p < 0.001). CONCLUSIONS:Classic baseline risk factors were more commonly observed in TAV aortopathy compared with BAV aortopathy. However, it is reassuring that, though diagnosed with aneurysm on average 10 years earlier and in the absence of arterial hypertension, BAV patients had a relatively low growth rate, similar to patients with a tricuspid valve. Irrespective of aortic valve morphology, patients with a family history of aortic aneurysm, history of coronary artery disease, and those who developed severe aortic regurgitation at follow-up, had the highest chances of being referred for surgery.
10.1016/j.yjmcc.2019.07.010
Histopathological changes in dilated ascending aorta associated with aortic valve cuspidity.
Karalko Mikita,Stejskal Vaclav,Dergel Martin,Gofus Jan,Timbilla Salifu,Zaloudkova Lenka,Zacek Pavel,Pojar Marek,Vojacek Jan
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
OBJECTIVES:Patients with a bicuspid aortic valve (BAV) often present with a dilated ascending aorta. However, the underlying pathogenesis for the observed changes in the aortic wall and the resulting aneurysmal dilation remains a subject of debate. This study aims to compare the histological abnormalities of the ascending aorta in BAV and tricuspid aortic valve (TAV) patients and their correlation with aortic diameter and patient age. METHODS:A total of 376 patients from our institution's clinical database were included in the retrospective analysis. These patients underwent either elective surgery for ascending aorta dilation or emergency surgery for aortic dissection, either isolated or with a structurally diseased aortic valve. After excision, the ascending aorta samples were analysed by a pathologist. RESULTS:On histological examination, a higher degree of elastic fibre fragmentation and loss and mucoid extracellular matrix accumulation was present in the samples from TAV patients when compared with that from BAV patients (P < 0.001). However, correlation was poor for all variables when considering aortic diameter and histological abnormalities or age and histological abnormalities in both BAV and TAV patients. CONCLUSIONS:Our study demonstrates a greater incidence of severe histological abnormalities in TAV patients when compared with BAV patients.
10.1093/ejcts/ezaa440
Outcomes of Bicuspid Aortic Valve Thoracic Aorta (4.0-4.5 cm) After Aortic Valve Replacement.
The Annals of thoracic surgery
BACKGROUND:There is little evidence on managing the proximal aorta of 4.0-4.5 cm during aortic valve replacement (AVR) in bicuspid aortic valve patients. METHODS:A total of 431 patients between 1993 and 2019 underwent either an isolated AVR, AVR + concomitant ascending aorta replacement, or aortic root replacement. We divided patients into native root dilation (4.0-4.5 cm, n = 121) vs root control groups (<4.0 cm, n = 238), native ascending dilation (4.0-4.5 cm, n = 50) vs ascending control groups (<4.0 cm, n = 166), or proximal dilation (root or ascending aorta 4.0-4.5 cm, n = 160) and proximal control groups (both root and ascending aorta <4.0 cm, n = 272). RESULTS:Growth rate was similar between the root dilation and control groups, (both were 0.1 mm/y, P = .56). The ascending dilation group had an aorta growth rate of 0.0 mm/y after AVR or root replacement, which was significantly different from the ascending control group (0.2 mm/y), P = .01. Furthermore, growth rate was similar between the proximal dilation (combined root or ascending dilation) and control group (both were 0.1 mm/y, P = .20). There were only 2 ascending aortic aneurysm repairs after AVR in the whole cohort. The long-term survival was similar between the root or ascending dilation groups vs root or ascending control groups, and between the proximal dilation and control groups. Multivariable Cox regression confirmed aortic root or ascending dilation was not a significant risk factor of long-term mortality. CONCLUSIONS:Our findings supported not replacing a 4.0-4.5 cm proximal thoracic aorta, including aortic root and ascending aorta, at the time of AVR for bicuspid aortic valve patients.
10.1016/j.athoracsur.2021.05.078
Machine-learning phenotypic classification of bicuspid aortopathy.
Wojnarski Charles M,Roselli Eric E,Idrees Jay J,Zhu Yuanjia,Carnes Theresa A,Lowry Ashley M,Collier Patrick H,Griffin Brian,Ehrlinger John,Blackstone Eugene H,Svensson Lars G,Lytle Bruce W
The Journal of thoracic and cardiovascular surgery
BACKGROUND:Bicuspid aortic valves (BAV) are associated with incompletely characterized aortopathy. Our objectives were to identify distinct patterns of aortopathy using machine-learning methods and characterize their association with valve morphology and patient characteristics. METHODS:We analyzed preoperative 3-dimensional computed tomography reconstructions for 656 patients with BAV undergoing ascending aorta surgery between January 2002 and January 2014. Unsupervised partitioning around medoids was used to cluster aortic dimensions. Group differences were identified using polytomous random forest analysis. RESULTS:Three distinct aneurysm phenotypes were identified: root (n = 83; 13%), with predominant dilatation at sinuses of Valsalva; ascending (n = 364; 55%), with supracoronary enlargement rarely extending past the brachiocephalic artery; and arch (n = 209; 32%), with aortic arch dilatation. The arch phenotype had the greatest association with right-noncoronary cusp fusion: 29%, versus 13% for ascending and 15% for root phenotypes (P < .0001). Severe valve regurgitation was most prevalent in root phenotype (57%), followed by ascending (34%) and arch phenotypes (25%; P < .0001). Aortic stenosis was most prevalent in arch phenotype (62%), followed by ascending (50%) and root phenotypes (28%; P < .0001). Patient age increased as the extent of aneurysm became more distal (root, 49 years; ascending, 53 years; arch, 57 years; P < .0001), and root phenotype was associated with greater male predominance compared with ascending and arch phenotypes (94%, 76%, and 70%, respectively; P < .0001). Phenotypes were visually recognizable with 94% accuracy. CONCLUSIONS:Three distinct phenotypes of bicuspid valve-associated aortopathy were identified using machine-learning methodology. Patient characteristics and valvular dysfunction vary by phenotype, suggesting that the location of aortic pathology may be related to the underlying pathophysiology of this disease.
10.1016/j.jtcvs.2017.08.123
Mid-term follow-up of aortic valve replacement for bicuspid aortic valve.
Liu Lingchao,Li Tianbo,Xu Bo,Liu Chencheng,Tang Fuqin,Xiao Yingbin,Wang Yong
Cardiology in the young
OBJECTIVE:The purpose of this study was to evaluate the mid-term outcome of aortic valve replacement for bicuspid aortic valve and tricuspid aortic valve and the related risk factors. METHODS:From January 2014 to June 2019, 177 tricuspid aortic valve patients and 101 bicuspid aortic valve patients who underwent aortic valve replacement in our hospital were collected. 1:1 propensity score matching analysis was used to control the bias in patient selection. The perioperative and follow-up data between the two groups were compared. Independent risk factors which were associated with the continued dilatation of the ascending aorta were identified by univariate or multivariate logistic regression analysis. RESULTS:After the matching procedure, 160 patients were included in the analysis (80 in each group). Baseline characteristics, intraoperative, and perioperative outcomes were similar between the two groups (all p > 0.05). Moreover, 67 patients in the tricuspid aortic valve group and 70 in the bicuspid aortic valve group completed the follow-up. The ascending aorta change, annual change rate, and the proportion of continuous dilation of ascending aorta in bicuspid aortic valve group were significantly higher than those in the tricuspid aortic valve group (p < 0.05). Multivariate logistic regression analysis showed that type 1 in bicuspid aortic valve (OR 5.173; 95% CI 1.772, 15.101; p = 0.003), aortic regurgitation (OR 3.673; 95% CI 1.133, 11.908; p = 0.030), and aortic valve stenosis with regurgitation (OR 6.489; 95% CI 1.726, 24.404; p = 0.006) were independent risk factors for the continued dilatation of the ascending aorta in all AV patients. Furthermore, the multivariate logistic regression analysis showed that type 1 in bicuspid aortic valve (OR 5.157; 95% CI 1.053, 25.272; p = 0.043), age ≥ 40 years (OR 6.956; 95% CI 1.228, 39.410; p = 0.028), and aortic regurgitation (OR 4.322; 95% CI 1.174, 15.911; p = 0.028) were independent risk factors for the continued dilatation of the ascending aorta in bicuspid aortic valve patients. CONCLUSION:Compared with tricuspid aortic valve patients, the ascending aorta of bicuspid aortic valve patients is more likely to continue to enlarge after aortic valve replacement. Type 1 in bicuspid aortic valve, age ≥ 40 years, and aortic regurgitation were the independent risk factors.
10.1017/S1047951121000160
Bicuspid aortic valve aortopathies: An hemodynamics characterization in dilated aortas.
Oliveira Diana,Rosa Sílvia Aguiar,Tiago Jorge,Ferreira Rui Cruz,Agapito Ana Figueiredo,Sequeira Adélia
Computer methods in biomechanics and biomedical engineering
Bicuspid aortic valve (BAV) aortopathy remains of difficult clinical management due to its heterogeneity and further assessment of related aortic hemodynamics is necessary. The aim of this study was to assess systolic hemodynamic indexes and wall stresses in patients with diverse BAV phenotypes and dilated ascending aortas. The aortic geometry was reconstructed from patient-specific images while the aortic valve was generated based on patient-specific measurements. Physiologic material properties and boundary conditions were applied and fully coupled fluid-structure interaction (FSI) analysis were conducted. Our dilated aortic models were characterized by the presence of abnormal hemodynamics with elevated degrees of flow skewness and eccentricity, regardless of BAV morphotype. Retrograde flow was also present. Both features, predicted by flow angle and flow reversal ratios, were consistently higher than those reported for non-dilated aortas. Right-handed helical flow was present, as well as elevated wall shear stress (WSS) on the outer ascending aortic wall. Our results suggest that the abnormal flow associated with BAV may play a role in aortic enlargement and progress it further on already dilated aortas.
10.1080/10255842.2019.1597860
Identification of hemodynamic biomarkers for bicuspid aortic valve induced aortic dilation using machine learning.
Franco Pamela,Sotelo Julio,Guala Andrea,Dux-Santoy Lydia,Evangelista Arturo,Rodríguez-Palomares José,Mery Domingo,Salas Rodrigo,Uribe Sergio
Computers in biology and medicine
Recent advances in medical imaging have confirmed the presence of altered hemodynamics in bicuspid aortic valve (BAV) patients. Therefore, there is a need for new hemodynamic biomarkers to refine disease monitoring and improve patient risk stratification. This research aims to analyze and extract multiple correlation patterns of hemodynamic parameters from 4D Flow MRI data and find which parameters allow an accurate classification between healthy volunteers (HV) and BAV patients with dilated and non-dilated ascending aorta using machine learning. Sixteen hemodynamic parameters were calculated in the ascending aorta (AAo) and aortic arch (AArch) at peak systole from 4D Flow MRI. We used sequential forward selection (SFS) and principal component analysis (PCA) as feature selection algorithms. Then, eleven machine-learning classifiers were implemented to separate HV and BAV patients (non- and dilated ascending aorta). Multiple correlation patterns from hemodynamic parameters were extracted using hierarchical clustering. The linear discriminant analysis and random forest are the best performing classifiers, using five hemodynamic parameters selected with SFS (velocity angle, forward velocity, vorticity, and backward velocity in AAo; and helicity density in AArch) a 96.31 ± 1.76% and 96.00 ± 0.83% accuracy, respectively. Hierarchical clustering revealed three groups of correlated features. According to this analysis, we observed that features selected by SFS have a better performance than those selected by PCA because the five selected parameters were distributed according to 3 different clusters. Based on the proposed method, we concluded that the feature selection method found five potentially hemodynamic biomarkers related to this disease.
10.1016/j.compbiomed.2021.105147
Surgical management of the aorta in BAV patients.
Etz Christian D,Haunschild Josephina,Girdauskas Evaldas,Della Corte Alessandro,Fedak Paul W M,Schäfers Hans-Joachim,Sundt Thoralf M,Borger Michael A
Progress in cardiovascular diseases
Patients with a bicuspid aortic valve (BAV) frequently develop aneurysms of the aortic root and tubular ascending aorta. Aneurysms of the aortic arch, in the absence of concomitant aortopathies, are much less common. According to the 2018 American Association of Thoracic Surgery consensus guidelines on BAV-related aortopathy, prophylactic surgical aortic repair / replacement is recommended starting at a maximum aortic diameter of 50 mm in patients with risk factors. Concomitant aortic surgery is also recommended at an aortic diameter of 45 mm in those patients with other indications for cardiac surgery (most commonly aortic valve procedures). The ultimate goal of prophylactic aortic surgery is the prevention of aortic catastrophes, e.g. aortic rupture or acute aortic dissection, which are associated with high morbidity and mortality. The surgical technique used - in elective and emergency cases - depends on the involvement and nature of the aortic valve disease, as well as the extent of aortic aneurysm formation. The current review focusses on the surgical management of the aortic root, tubular ascending aorta, and proximal aortic arch in BAV patients. Despite the abovementioned recommendations, many BAV patients develop acute aortic syndromes below the recommended aortic diameter thresholds. Further research is therefore required in order to identify high-risk BAV subgroups that would benefit from earlier surgical repair.
10.1016/j.pcad.2020.06.013
Transcatheter aortic valve replacement in bicuspid aortic valve stenosis: where do we stand?
Yoon Sung-Han,Sharma Rahul,Chakravarty Tarun,Miyasaka Masaki,Ochiai Tomoki,Nomura Takahiro,Gellada Norman,Nemanpour Shadi,Nakamura Mamoo,Chen Wen,Makkar Raj
The Journal of cardiovascular surgery
Bicuspid aortic valve is the most common congenital cardiac defect in adults, and symptom typically develops in adulthood. In the majority of cases, bicuspid aortic valve disease progress with ages and surgical aortic valve replacement is performed with excellent operative outcomes. However, with the relatively slow progression of disease, surgical aortic valve replacement is required in elderly patients but the surgical risk often deemed extremely high due to old age and multiple comorbidities. Transcatheter aortic valve replacement (TAVR) has evolved from a novel technology to an established therapy for intermediate- and high-risk patients with symptomatic severe aortic valve stenosis (AS). Numerous studies have demonstrated the safety and efficacy of TAVR, and more than 250,000 patients have been treated with this technology. Although randomized trials have established TAVR as the standard treatment, these trials excluded congenital bicuspid AS due to its unique morphological features. Nevertheless, the growing experience, accumulated knowledge, and advancements of new technology lead to the expand use of TAVR to other pathologies or other populations such as bicuspid AS. With integration of imaging multimodalities (computed tomography and echocardiography), the diagnosis and classification of bicuspid aortic valve has been changing. Due to unfavorable anatomic features of bicuspid AS, the outcomes of TAVR in bicuspid AS was suboptimal, particularly when using the first-generation transcatheter valves. However, the newer-generation transcatheter valves significantly improved the outcomes of TAVR in bicuspid AS. Nonetheless, several issues still remain to be resolved. Given longer life expectancy in patients with bicuspid AS undergoing TAVR, durability of transcatheter valves is concerned. In addition, patients with bicuspid aortic valves often have concomitant dilatation of proximal part of ascending aorta (aortopathy), but limited data exist about the clinical prognosis of bicuspid aortic valve with concomitant aortopathy in elderly patients. Considering the expanding indication of TAVR to lower surgical risk and younger population, these issues should be evaluated in future studies.
10.23736/S0021-9509.18.10350-8
Bicuspid aortic valve and aortopathy: novel prognostic predictors for the identification of high-risk patients.
Longobardo Luca,Carerj Scipione,Bitto Alessandra,Cusmà-Piccione Maurizio,Carerj Maria Ludovica,Calabrò Maria Pia,Di Bella Gianluca,Licordari Roberto,Squadrito Francesco,Khandheria Bijoy K,Zito Concetta
European heart journal. Cardiovascular Imaging
AIMS:Bicuspid aortic valve (BAV) may be complicated by aortic aneurysms and dissection. This study aimed to evaluate the prognostic efficacy of markers from cardiac imaging, as well as genetic and new biomarkers, to early predict aortic complications. METHODS AND RESULTS:We re-evaluated after a mean time of 48 ± 11 months 47 BAV patients who had undergone previous echocardiography for evaluation of aortic stiffness and 2D aortic longitudinal strain (LS) (by speckle-tracking analysis), and who had given a blood sample for the assessment of a single-nucleotide polymorphism of elastin gene (ELN rs2 071307) and quantification of elastin soluble fragments (ESF). Surgical treatment of aortic aneurysm/dissection was the primary endpoint, and an aortic dimension increase (of one or more aortic segments) ≥1 mm/year was the secondary endpoint. Nine patients underwent surgical treatment of ascending aorta (AA) aneurysms. Out of the 38 patients who did not need surgical intervention, 16 showed an increase of aortic root and/or AA dimension ≥1 mm/year. At multivariate Cox regression analysis, an impaired AA LS was an independent predictor of aortic surgery [P = 0.04; hazard ratio (HR) 0.961; 95% confidence interval (CI) 0.924-0.984] and aortic dilatation (P = 0.007; HR 0.960; 95% CI 0.932-0.989). An increased quantity of ESF was correlated (P = 0.015) with the primary endpoint at univariate Cox regression analysis but it did not keep statistical significance at multivariate analysis. CONCLUSION:In BAV patients, impairment of elastic properties of the AA, as assessed by 2D LS, is an effective predictor of aortic complications.
10.1093/ehjci/jeaa250
Bicuspid aortic valve aortopathy is characterized by embryonic epithelial to mesenchymal transition and endothelial instability.
Journal of molecular medicine (Berlin, Germany)
Bicuspid aortic valve (BAV) is the most common congenital heart malformation frequently associated with ascending aortic aneurysm (AscAA). Epithelial to mesenchymal transition (EMT) may play a role in BAV-associated AscAA. The aim of the study was to investigate the type of EMT associated with BAV aortopathy using patients with a tricuspid aortic valve (TAV) as a reference. The state of the endothelium was further evaluated. Aortic biopsies were taken from patients undergoing open-heart surgery. Aortic intima/media miRNA and gene expression was analyzed using Affymetrix human transcriptomic array. Histological staining assessed structure, localization, and protein expression. Migration/proliferation was assessed using ORIS migration assay. We show different EMT types associated with BAV and TAV AscAA. Specifically, in BAV-associated aortopathy, EMT genes related to endocardial cushion formation were enriched. Further, BAV vascular smooth muscle cells were less proliferative and migratory. In contrast, TAV aneurysmal aortas displayed a fibrotic EMT phenotype with medial degenerative insults. Further, non-dilated BAV aortas showed a lower miRNA-200c-associated endothelial basement membrane LAMC1 expression and lower CD31 expression, accompanied by increased endothelial permeability indicated by increased albumin infiltration. Embryonic EMT is a characteristic of BAV aortopathy, associated with endothelial instability and vascular permeability of the non-dilated aortic wall. KEY MESSAGES: Embryonic EMT is a feature of BAV-associated aortopathy. Endothelial integrity is compromised in BAV aortas prior to dilatation. Non-dilated BAV ascending aortas are more permeable than aortas of tricuspid aortic valve patients.
10.1007/s00109-023-02316-5
Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease.
Evangelista Arturo,Gallego Pastora,Calvo-Iglesias Francisco,Bermejo Javier,Robledo-Carmona Juan,Sánchez Violeta,Saura Daniel,Arnold Roman,Carro Amelia,Maldonado Giuliana,Sao-Avilés Augusto,Teixidó Gisela,Galian Laura,Rodríguez-Palomares José,García-Dorado David
Heart (British Cardiac Society)
OBJECTIVE:Bicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications. METHODS:Eight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention. BAV morphotype, significant valve dysfunction and aorta dilation (≥2 Z-score) at sinuses and ascending aorta were established. RESULTS:Three BAV morphotypes were identified: right-left coronary cusp fusion (RL) in 72.9%, right-non-coronary (RN) in 24.1% and left-non-coronary (LN) in 3.0%. BAV without raphe was observed in 18.3%. Multivariate analysis showed aortic regurgitation (23%) to be related to male sex (OR: 2.80, p<0.0001) and valve prolapse (OR: 5.16, p<0.0001), and aortic stenosis (22%) to BAV-RN (OR: 2.09, p<0.001), the presence of raphe (OR: 2.75, p<0.001), age (OR: 1.03; p<0.001), dyslipidaemia (OR: 1.77, p<0.01) and smoking (OR: 1.63, p<0.05). Ascending aorta was dilated in 76% without differences among morphotypes and associated with significant valvular dysfunction. By contrast, aortic root was dilated in 34% and related to male sex and aortic regurgitation but was less frequent in aortic stenosis and BAV-RN. CONCLUSIONS:Normofunctional valves are more prevalent in BAV without raphe. Aortic stenosis is more frequent in BAV-RN and associated with some cardiovascular risk factors, whereas aortic regurgitation (AR) is associated with male sex and sigmoid prolapse. Although ascending aorta is the most commonly dilated segment, aortic root dilation is present in one-third of patients and associated with AR. Remarkably, BAV-RL increases the risk for dilation of the proximal aorta, whereas BAV-RN spares this area.
10.1136/heartjnl-2017-311560
Bicuspid Aortic Valves: an Up-to-Date Review on Genetics, Natural History, and Management.
Current cardiology reports
PURPOSE OF REVIEW:Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. It has a wide spectrum of clinical manifestations including aortic regurgitation (AR), aortic stenosis, and an associated aortopathy with a small but increased risk of aortic dissection. This review describes current knowledge of BAV, from anatomy and genetics to a discussion of multifaceted strategies utilized in the management of this unique patient population. This review will also highlight critical knowledge gaps in areas of basic and clinical research to enhance further understanding of this clinical entity. RECENT FINDINGS:The current knowledge regarding pathophysiologic mechanisms, screening, and surveillance guidelines for BAV and the associated aortopathy is discussed. We also discuss current management techniques for aortic valve repair versus replacement, indications for aortic surgery (root or ascending aorta), and the emergence of the Ross procedure as a viable management option not only in children, but also in adolescents and adults. The varied clinical phenotype of the BAV, resulting in its specific complex hemodynamic interactions, renders it an entity which is separate and distinct from the tricuspid aortic valve pathologies. While various aortic histopathologic and protein alterations in BAV patients have been described, it remains unclear if these changes are causal or the result of hemodynamic alterations imposed by sheer stress on the intrinsically dysfunctional BAV. Medical management for patients with BAV with AS, AI, or dilated aortic roots/ascending aortas remains challenging and needs further investigation. More than 50% of patients with BAV will undergo AVR during their lifetime, and more than 25% of patients with BAV undergo aortic surgery performed for dilation of the aortic root or ascending aorta, often concurrently with AVR. The search for the ultimate genetic or epigenetic cause of the different bicuspid phenotypes will ultimately be facilitated by the next-generation sequencing tools that allow for study of large populations at low cost. Improvements in diagnostic and stratification criteria to accurately risk assess BAV patients are critical to this process.
10.1007/s11886-022-01716-2
Ascending aortic wall degeneration in patients with bicuspid versus tricuspid aortic valve.
Journal of cardiothoracic surgery
BACKGROUND:The magnitude of ascending aortic degeneration in patients with bicuspid aortic valves (BAV) is controversial. METHODS:The aim of this study was to investigate ascending aortic wall degeneration in patients with BAV as compared with tricuspid aortic valves (TAV). The ascending aortic wall of 67 consecutive patients was processed for histology and immunohistochemistry. The extent of surgery and wall degeneration were investigated. Unadjusted survival was evaluated by Kaplan-Meier analysis. Median follow-up for patients with BAV and TAV was 3.8 years (interquartile range [IQR] 3.5-4.1) and 3.7 years (IQR 3.4-3.9), respectively. RESULTS:There were 33 patients with BAV and 34 with TAV. Mid-ascending aorta diameter was 54 mm (IQR 50-60). Replacement of the aortic valve, together with an ascending aortic prosthesis, was more frequent in BAV vs TAV patients (24% vs. 3%, P = 0.013). However, medial fibrosis, elastic fiber thinning, incremental medial degeneration and smooth muscle cell nuclei loss were less prominent in BAV vs TAV patients (0.1 ± 0.4 vs. 0.8 ± 1.4, P = 0.016; 0.6 ± 1.4 vs. 1.6 ± 2.0, P = 0.027; 1.7 ± 0.7 vs. 2.2 ± 0.8, P = 0.045 and 2.3 ± 1.5 vs. 3.2 ± 1.3, P = 0.026, respectively). CONCLUSIONS:Since degeneration of the ascending aortic wall was seldom prominent, histopathology alone may not support the need for surgery of the dilated ascending aorta in BAV patients as compared with TAV patients.
10.1186/s13019-022-01864-0
Bicuspid aortic valve-associated aortopathy: Where do we stand?
Messner Barbara,Bernhard David
Journal of molecular and cellular cardiology
Herein we summarize the current knowledge on the bicuspid aortic valve (BAV)-associated aortopathy regarding clinical presentation and disease sub-classification, genetic background, hemodynamics, histopathology, cells and signaling, animal models, and biomarkers. Despite enormous efforts in research in all of the above areas, important issues remain unknown: (i) what is the ontogenetic basis of BAV development? (ii) how can we explain the diversity of BAV and associated aortopathy phenotypes? (iii) what are the signaling processes in aortopathy pathogenesis and how can we interfere with these processes? Despite undoubtedly great progress that has been made in the understanding of BAV-associated aortopathy, so far researchers have put together a heap of Lego bricks, but at present it is unclear if the bricks are compatible, how they fit together, and which parts are missing to build the true model of the BAV aorta. A joint approach is needed to accelerate research progress.
10.1016/j.yjmcc.2019.05.023
Association of Regional Wall Shear Stress and Progressive Ascending Aorta Dilation in Bicuspid Aortic Valve.
JACC. Cardiovascular imaging
OBJECTIVES:The aim of this study was to evaluate the role of wall shear stress (WSS) as a predictor of ascending aorta (AAo) growth at 5 years or greater follow-up. BACKGROUND:Aortic 4-dimensional flow cardiac magnetic resonance (CMR) can quantify regions exposed to high WSS, a known stimulus for arterial wall dysfunction. However, its association with longitudinal changes in aortic dilation in patients with bicuspid aortic valve (BAV) is unknown. METHODS:This retrospective study identified 72 patients with BAV (age 45 ± 12 years) who underwent CMR for surveillance of aortic dilation at baseline and ≥5 years of follow-up. Four-dimensional flow CMR analysis included the calculation of WSS heat maps to compare regional WSS in individual patients with population averages of healthy age- and sex-matched subjects (database of 136 controls). The relative areas of the AAo and aorta (in %) exposed to elevated WSS (outside the 95% CI of healthy population averages) were quantified. RESULTS:At a median follow-up duration of 6.0 years, the mean AAo growth rate was 0.24 ± 0.20 mm/y. The fraction of the AAo exposed to elevated WSS at baseline was increased for patients with higher growth rates (>0.24 mm/y, n = 32) compared with those with growth rates <0.24 mm/y (19.9% [IQR: 10.2%-25.5%] vs 5.7% [IQR: 1.5%-21.3%]; P = 0.008). Larger areas of elevated WSS in the AAo and entire aorta were associated with higher rates of AAo dilation >0.24 mm/y (odds ratio: 1.51; 95% CI: 1.05-2.17; P = 0.026 and odds ratio: 1.70; 95% CI: 1.01-3.15; P = 0.046, respectively). CONCLUSIONS:The area of elevated AAo WSS as assessed by 4-dimensional flow CMR identified BAV patients with higher rates of aortic dilation and thus might determine which patients require closer follow-up.
10.1016/j.jcmg.2021.06.020
Aortic wall thickness in dilated ascending aorta: Comparison between tricuspid and bicuspid aortic valve.
Archives of cardiovascular diseases
BACKGROUND:Bicuspid aortic valve (BAV) is frequently associated with dilatation of the thoracic aorta. Peculiar anatomical, histological and mechanical changes of the aortic wall in BAV aortopathy have been hypothesized to suggest an increased risk of acute aortic complications in patients with BAV. AIM:In this study we tried to clarify any differences in the adaptability of the aortic wall to the mechanism of dilatation between patients with BAV and those with TAV. METHODS:In total, 354 samples were taken from 71 patients undergoing elective aortic surgery and divided into two groups: BAV group (n=16; 101 samples); and TAV group (n=55; 253 samples). Aortic wall thickness was measured with a dedicated caliper. The relationship between aortic wall thickness and aortic dilatation and demographic variables was evaluated cumulatively and comparatively (BAV versus TAV). In patients with more than three samples available, intrapatient variability was also studied. Finally, potential risk factors for severely reduced aortic wall thickness were also assessed. RESULTS:Analysis of preoperative characteristics revealed significant differences in patient age (54±16years for BAV and 66±11years for TAV; P=0.0011), with no differences in variables related to aortic dilatation (including phenotype). Cumulative aortic wall thickness was significantly thinner in the anterior than in the posterior wall. In the comparative analysis, aortic wall thickness was significantly thinner in patients with BAV in both the anterior and posterior regions. Furthermore, in patients with BAV, dilatation>51mm was a significant predictor of severely reduced aortic wall thickness. CONCLUSIONS:In our experience, patients with BAV aortopathy reached the cut-off for the surgical indication at an early age. Careful monitoring in patients with BAV is mandatory when aortic dilatation has reached 51mm, as it is related to significant anatomical changes.
10.1016/j.acvd.2023.08.003
Bicuspid Aortic Valve and Endothelial Dysfunction: Current Evidence and Potential Therapeutic Targets.
Antequera-González Borja,Martínez-Micaelo Neus,Alegret Josep M
Frontiers in physiology
Bicuspid aortic valve (BAV), the most frequent congenital heart malformation, is characterized by the presence of a two-leaflet aortic valve instead of a three-leaflet one. BAV disease progression is associated with valvular dysfunction (in the form of stenosis or regurgitation) and aortopathy, which can lead to aneurysm and aortic dissection. This morphological abnormality modifies valve dynamics and promotes eccentric blood flow, which gives rise to alterations of the flow pattern and wall shear stress (WSS) of the ascending aorta. Recently, evidence of endothelial dysfunction (ED) in BAV disease has emerged. Different studies have addressed a reduced endothelial functionality by analyzing various molecular biomarkers and cellular parameters in BAV patients. Some authors have found impaired functionality of circulating endothelial progenitors in these patients, associating it with valvular dysfunction and aortic dilation. Others focused on systemic endothelial function by measuring artery flow-mediated dilation (FMD), showing a reduced FMD in BAV individuals. Novel biomarkers like increased endothelial microparticles (EMP), which are related to ED, have also been discovered in BAV patients. Finally, latest studies indicate that in BAV, endothelial-to-mesenchymal transition (EndoMT) may also be de-regulated, which could be caused by genetic, hemodynamic alterations, or both. Different hypothesis about the pathology of ED in BAV are nowadays being debated. Some authors blamed this impaired functionality just on genetic abnormalities, which could lead to a pathological aorta. Nevertheless, thanks to the development of new and high-resolution imaging techniques like 4D flow MRI, hemodynamics has gained great attention. Based on latest studies, alterations in blood flow seem to cause proper modification of the endothelial cells (ECs) function and morphology. It also seems to be associated with aortic dilation and decreased vasodilators expression, like nitric oxide (NO). Although nowadays ED in BAV has been reported by many, it is not clear which its main cause may be. Comprehending the pathways that promote ED and its relevance in BAV could help further understand and maybe prevent the serious consequences of this disease. This review will discuss the ED present in BAV, focusing on the latest evidence, biomarkers for ED and potential therapeutic targets (Figure 1).
10.3389/fphys.2020.01015
Serial echocardiography for valve dysfunction and aortic dilation in bicuspid aortic valves.
Parker Matthew W,Muchnik Renee Dallasen,Ogunsua Adedotun,Kundu Amartya,Lakshmanan Suvasini,Shah Nikhil,Dickey John B,Gentile Bryon A,Pape Linda A
Echocardiography (Mount Kisco, N.Y.)
BACKGROUND AND AIMS:Complications of bicuspid aortic valve commonly include aortic stenosis, aortic regurgitation, and ascending aortic dilation. The progression of these lesions is not well described. MATERIALS AND METHODS:We reviewed 249 bicuspid aortic valve patients with at least two echocardiograms from 2006 to 2016. Valve morphology (right-left or right-noncoronary cusp fusion) was confirmed by visual inspection, and aortic stenosis and regurgitation were quantified according to current guidelines; the ascending aorta was measured at end-systole 2-3 cm above the sinotubular junction. Annualized progression of stenosis, regurgitation, and aortic dilation from first to most recent echocardiogram were compared between right-left and right-nonfused valves using multivariable logistic regression to adjust for baseline differences in groups. RESULTS:Among 249 bicuspid aortic valve patients (mean age 47.6 ± 13.5 years, 66.3% male), 75.9% had right-left cusp fusion. At baseline, aortic stenosis was absent or mild in 80.3%; aortic regurgitation was absent or mild in 80.7%; and aortic diameters were 35.0 ± 5.7 mm (sinuses of Valsalva) and 37.4 ± 6.2 mm (ascending). Mean annualized decrease in aortic valve area was 0.07 cm /year, with 30% of bicuspid aortic valve patients progressing ≥0.1 cm /year. Aortic regurgitation progressed ≥1 grade in 37 patients. Mean annualized increase in ascending aorta diameter was 0.36 mm/year in right-left and 0.65 mm/year in right-nonbicuspid valves. CONCLUSIONS:In this serial echocardiographic study of bicuspid aortic valve patients, cusp orientation was not associated with progression of valve dysfunction. Right-noncoronary cusp fusion was associated with ascending aortic diameter progression.
10.1111/echo.15000
Bicuspid aortic valve outcomes.
Rodrigues Inês,Agapito Ana F,de Sousa Lídia,Oliveira José A,Branco Luísa M,Galrinho Ana,Abreu João,Timóteo Ana T,Rosa Sílvia A,Ferreira Rui C
Cardiology in the young
BACKGROUND:Bicuspid aortic valve is the most common CHD. Its association with early valvular dysfunction, endocarditis, thoracic aorta dilatation, and aortic dissection is well established. OBJECTIVE:The aim of this study was to assess the incidence and predictors of cardiac events in adults with bicuspid aortic valve. METHODS:We carried out a retrospective analysis of cardiac outcomes in ambulatory adults with bicuspid aortic valve followed-up in a tertiary hospital centre. Outcomes were defined as follows: interventional - intervention on the aortic valve or thoracic aorta; medical - death, aortic dissection, aortic valve endocarditis, congestive heart failure, arrhythmias, or ischaemic heart disease requiring hospital admission; and a composite end point of both. Kaplan-Meier curves were generated to determine event rates, and predictors of cardiac events were determined by multivariate analysis. RESULTS:A total of 227 patients were followed-up over 13±9 years; 29% of patients developed severe aortic valve dysfunction and 12.3% reached ascending thoracic aorta dimensions above 45 mm. At least one cardiac outcome occurred in 38.8% of patients, with an incidence rate at 20 years of follow-up of 47±4%; 33% of patients were submitted to an aortic valve or thoracic aorta intervention. Survival 20 years after diagnosis was 94±2%. Independent predictors of the composite end point were baseline moderate-severe aortic valve dysfunction (hazard ratio, 3.19; 95% confidence interval, 1.35-7.54; p<0.01) and aortic valve leaflets calcification (hazard ratio, 4.72; 95% confidence interval, 1.91-11.64; p<0.005). CONCLUSIONS:In this study of bicuspid aortic valve, the long-term survival was excellent but with occurrence of frequent cardiovascular events. Baseline aortic valve calcification and dysfunction were the only independent predictors of events.
10.1017/S1047951116002560
Wall Shear Stress Directional Abnormalities in BAV Aortas: Toward a New Hemodynamic Predictor of Aortopathy?
Liu Janet,Shar Jason A,Sucosky Philippe
Frontiers in physiology
The bicuspid aortic valve (BAV) generates wall shear stress (WSS) abnormalities in the ascending aorta (AA) that may be responsible for the high prevalence of aortopathy in BAV patients. While previous studies have analyzed the magnitude and oscillatory characteristics of the total or streamwise WSS in BAV AAs, the assessment of the circumferential component is lacking despite its expected significance in this highly helical flow environment. This gap may have hampered the identification of a robust hemodynamic predictor of BAV aortopathy. The objective of this study was to perform a global and component-specific assessment of WSS magnitude, oscillatory and directional characteristics in BAV AAs. The WSS environments were computed in the proximal and middle convexity of tricuspid aortic valve (TAV) and BAV AAs using our previous valve-aorta fluid-structure interaction (FSI) models. Component-specific WSS characteristics were investigated in terms of temporal shear magnitude (TSM) and oscillatory shear index (OSI). WSS directionality was quantified in terms of mean WSS vector magnitude and angle, and angular dispersion index (D). Local WSS magnitude and multidirectionality were captured in a new shear magnitude and directionality index (SMDI) calculated as the product of the mean WSS magnitude and D. BAVs subjected the AA to circumferential TSM overloads (2.4-fold increase vs. TAV). TAV and BAV AAs exhibited a unidirectional circumferential WSS (OSI < 0.04) and an increasingly unidirectional longitudinal WSS between the proximal (OSI > 0.21) and middle (OSI < 0.07) sections. BAVs generated mean WSS vectors skewed toward the anterior wall and WSS angular distributions exhibiting decreased uniformity in the proximal AA (0.27-point increase in D vs. TAV). SMDI was elevated in all BAV AAs but peaked in the proximal LR-BAV AA (3.6-fold increase vs. TAV) and in the middle RN-BAV AA (1.6-fold increase vs. TAV). This analysis demonstrates the significance of the circumferential WSS component and the existence of substantial WSS directional abnormalities in BAV AAs. SMDI abnormality distributions in BAV AAs follow the morphotype-dependent occurrence of dilation in BAV AAs, suggesting the predictive potential of this metric for BAV aortopathy.
10.3389/fphys.2018.00993
Bicuspid aortic valve and its ascending aortopathy.
Current opinion in pediatrics
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.
10.1097/MOP.0000000000001276
Mechanisms of Aortic Dilation in Patients With Bicuspid Aortic Valve: JACC State-of-the-Art Review.
Journal of the American College of Cardiology
Bicuspid aortic valve is the most common congenital heart disease and exposes patients to an increased risk of aortic dilation and dissection. Aortic dilation is a slow, silent process, leading to a greater risk of aortic dissection. The prevention of adverse events together with optimization of the frequency of the required lifelong imaging surveillance are important for both clinicians and patients and motivated extensive research to shed light on the physiopathologic processes involved in bicuspid aortic valve aortopathy. Two main research hypotheses have been consolidated in the last decade: one supports a genetic basis for the increased prevalence of dilation, in particular for the aortic root, and the second supports the damaging impact on the aortic wall of altered flow dynamics associated with these structurally abnormal valves, particularly significant in the ascending aorta. Current opinion tends to rule out mutually excluding causative mechanisms, recognizing both as important and potentially clinically relevant.
10.1016/j.jacc.2022.10.042
Bicuspid aortic valve associated aortopathy: 2022 guideline update.
Current opinion in cardiology
PURPOSE OF REVIEW:Bicuspid aortic valve (BAV) disease is observed in 1-2% of the general population. In addition to valve-related 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 FINDINGS:The 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). SUMMARY:BAV 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.
10.1097/HCO.0000000000001020