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OBJECTIVES: The aims of the present study were to compare the long-term outcomes for ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement between patients with bicuspid aortic valve (BAV) and tricuspid aortic valve ( TAV). METHODS: This retrospective study included 310 patients who had undergone isolated aortic valve replacement with an ascending aorta diameter ≤ 45 mm between January 2010 and September 2021. The patients were divided into BAV group (n=90) and TAV group (n=220). The differences in the dilation rate of the ascending aorta and long-term outcomes were analyzed. RESULTS: Overall survival was 89 ± 4% in the BAV group vs. 75 ± 6% in the TAV group at 10 years postoperatively (P=0.007), yet this difference disappeared after adjusting exclusively for age (P=0.343). The mean annual growth rate of the ascending aorta was similar between the two groups during follow-up (0.5 ± 0.6 mm/year vs. 0.4 ± 0.5 mm/year; P=0.498). Ten-year freedom from adverse aortic events was 98.1% in the BAV group vs. 95.0% in the TAV group (P=0.636). Multivariable analysis revealed preoperative ascending aorta diameter to be a significant predictor of adverse aortic events (hazard ratio: 1.76; 95% confidence interval: 1.33 to 2.38; P<0.001). CONCLUSION: Our study revealed that the long-term survival and the risks of adverse aortic events between BAV and TAV patients were similar after isolated aortic valve replacement. BAV was not a risk factor of adverse aortic events.

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Abstract This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

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This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Este consenso de nomenclatura y clasificación para la válvula aórtica bicúspide congénita y su aortopatía está basado en la evidencia y destinado a ser utilizado universalmente por médicos (tanto pediatras como de adultos), médicos ecocardiografistas, especialistas en imágenes avanzadas cardiovasculares, cardiólogos intervencionistas, cirujanos cardiovasculares, patólogos, genetistas e investigadores que abarcan estas áreas de investigación clínica y básica. Siempre y cuando se disponga de nueva investigación clave y de referencia, este consenso internacional puede estar sujeto a cambios de acuerdo con datos basados en la evidencia1.

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Bicuspid aortic valve is the most common congenital heart defect. Transthoracic echocardiogram is the initial tool to assess and diagnose this condition, however, transesophageal echocardiogram with 3D modalities, including transillumination have a better anatomical and functional evaluation of the valve, allowing to classify the bicuspid aortic valve according to the position of the raphe and assess the main vessels for complications or exclude other cardiovascular diseases.

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ABSTRACT Objectives: The aims of the present study were to compare the long-term outcomes for ascending aortic dilatation and adverse aortic events after isolated aortic valve replacement between patients with bicuspid aortic valve (BAV) and tricuspid aortic valve ( TAV). Methods: This retrospective study included 310 patients who had undergone isolated aortic valve replacement with an ascending aorta diameter ≤ 45 mm between January 2010 and September 2021. The patients were divided into BAV group (n=90) and TAV group (n=220). The differences in the dilation rate of the ascending aorta and long-term outcomes were analyzed. Results: Overall survival was 89 ± 4% in the BAV group vs. 75 ± 6% in the TAV group at 10 years postoperatively (P=0.007), yet this difference disappeared after adjusting exclusively for age (P=0.343). The mean annual growth rate of the ascending aorta was similar between the two groups during follow-up (0.5 ± 0.6 mm/year vs. 0.4 ± 0.5 mm/year; P=0.498). Ten-year freedom from adverse aortic events was 98.1% in the BAV group vs. 95.0% in the TAV group (P=0.636). Multivariable analysis revealed preoperative ascending aorta diameter to be a significant predictor of adverse aortic events (hazard ratio: 1.76; 95% confidence interval: 1.33 to 2.38; P<0.001). Conclusion: Our study revealed that the long-term survival and the risks of adverse aortic events between BAV and TAV patients were similar after isolated aortic valve replacement. BAV was not a risk factor of adverse aortic events.

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The bicuspid aortic valve corresponds to the most common valve defect in the general population. This can present asymptomatically as an incidental finding or symptomatically due to valve disease (stenosis and regurgitation), aortopathy, and infective endocarditis. Imaging techniques such as transthoracic and transesophageal echocardiography are fundamental pillars in diagnosis and treatment, determining the need for surgical intervention and the type of surgery to be performed. The case of a young patient with a less frequent bicuspid aortic valve phenotype is presented.

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PURPOSE: To evaluate the clinical and surgical outcomes among children with bicuspid aortic valve who underwent cardiac surgery. METHODS: This observational and retrospective study included patients with a diagnosis of bicuspid aortic valve who underwent cardiac surgery between January 1, 2003, and March 31, 2020. Demographic characteristics and perioperative conditions were described. RESULTS: One hundred and sixteen patients were included, with a mean age of 12.4 ± 4.2 years; 63.2% were male. The most frequent diagnosis was congenital aortic stenosis (23.5%), followed by connective tissue disorders (16%). Mechanical aortic prostheses were used in 87.7% of cases, with a mean size of 21 ± 2.6 mm. The main factors associated with mortality were valve prosthesis dysfunction (odds ratio [OR]: 12.44; 95% confidence interval [CI]: 1.05-147.48; p = .04) and reoperation (OR: 24.29; 95% CI: 1.03-570.08; p = .04). The overall survival was 87.9%, with better outcomes among those who did not undergo reoperation (Log Rank, p = .01). CONCLUSIONS: Outcomes after aortic valve replacement in children with bicuspid aortic valve are excellent in the short and long term, regardless of using mechanical or biological prostheses.

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This case report shows how to perform simultaneously an open surgical correction of an aortic root aneurysm and aortic stenosis by interposition of an aortic composite graft and the transdiaphragmatic extra-anatomical correction of an aortic coarctation.

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Background and Purpose: Prognostic models based on cardiovascular hemodynamic parameters may bring new information for an early assessment of patients with bicuspid aortic valve (BAV), playing a key role in reducing the long-term risk of cardiovascular events. This work quantifies several three-dimensional hemodynamic parameters in different patients with BAV and ranks their relationships with aortic diameter. Materials and Methods: Using 4D-flow CMR data of 74 patients with BAV (49 right-left and 25 right-non-coronary) and 48 healthy volunteers, aortic 3D maps of seventeen 17 different hemodynamic parameters were quantified along the thoracic aorta. Patients with BAV were divided into two morphotype categories, BAV-Non-AAoD (where we include 18 non-dilated patients and 7 root-dilated patients) and BAV-AAoD (where we include the 49 patients with dilatation of the ascending aorta). Differences between volunteers and patients were evaluated using MANOVA with Pillai's trace statistic, Mann-Whitney U test, ROC curves, and minimum redundancy maximum relevance algorithm. Spearman's correlation was used to correlate the dilation with each hemodynamic parameter. Results: The flow eccentricity, backward velocity, velocity angle, regurgitation fraction, circumferential wall shear stress, axial vorticity, and axial circulation allowed to discriminate between volunteers and patients with BAV, even in the absence of dilation. In patients with BAV, the diameter presented a strong correlation (> |+/-0.7|) with the forward velocity and velocity angle, and a good correlation (> |+/-0.5|) with regurgitation fraction, wall shear stress, wall shear stress axial, and vorticity, also for morphotypes and phenotypes, some of them are correlated with the diameter. The velocity angle proved to be an excellent biomarker in the differentiation between volunteers and patients with BAV, BAV morphotypes, and BAV phenotypes, with an area under the curve bigger than 0.90, and higher predictor important scores. Conclusions: Through the application of a novel 3D quantification method, hemodynamic parameters related to flow direction, such as flow eccentricity, velocity angle, and regurgitation fraction, presented the best relationships with a local diameter and effectively differentiated patients with BAV from healthy volunteers.

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Percutaneous surgery is the treatment of choice of isolated aortic coarctation in adults However, when there are other heart problems related to aortic coarctation, its surgical management may vary. We report a 41-year-old male presenting with aortic coarctation associated with severe, symptomatic, bicuspid aortic valve lesions and significant left ventricular dysfunction. He underwent open heart surgery for the surgical resolution of these problems. One year after surgery the results are satisfactory with no evidence of postoperative complications and a significant improvement of patient symptoms and left ventricular function.

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Abstract Objectives: Bicuspid aortic valve (BAV) is an important aetiology of aortic stenosis and the use of transcatheter aortic valve implantation (TAVI) has not been fully explored in this cohort. This systematic review and meta-analysis compared the outcomes of TAVI in stenotic BAV against tricuspid aortic valve (TAV). Methods: An electronic literature search was performed in PubMed, MEDLINE, EMBASE, and Scopus to identify all studies comparing TAVI in stenotic BAV versus TAV. Only studies comparing TAVI in BAV versus TAV were included, without any limit on the study date. Primary endpoints were 30-day and 1-year mortality, while secondary endpoints were postoperative rates of stroke, acute kidney injury (AKI), and permanent pacemaker (PPM) requirement. A trial sequential analysis (TSA) was performed for all endpoints to understand their significance. Results: Thirteen studies met the inclusion criteria (917 BAV and 3079 TAV patients). The BAV cohort was younger (76.8±7.43 years vs. 78.5±7.12 years, P=0.02), had a higher trans-aortic valve gradient (P=0.02), and larger ascending aortic diameters (P<0.0001). No significant difference was shown for primary (30-day mortality [P=0.45] and 1-year mortality [P=0.41]) and secondary endpoints (postoperative stroke [P=0.49], AKI [P=0.14], and PPM requirement [P=0.86]). The BAV group had a higher rate of significant postoperative aortic regurgitation (P=0.002). TSA showed that there was sufficient evidence to conclude the lack of difference in PPM requirements, and 30-day and 1-year mortality between the two cohorts. Conclusion: TAVI gives satisfactory outcomes for treating stenotic BAV and should be considered clinically.

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OBJECTIVE: Surgery for ascending aneurysms in bicuspid aortic valve syndrome primarily includes Bentall root replacement, aortic valve replacement with supracoronary ascending aorta replacement (AVRSCAAR), and valve-sparing root reimplantation (VSRR). Comparative analysis of long-term clinical and functional outcomes of these procedures is detailed. METHODS: From 1997 to 2017, 635 patients with bicuspid aortic valve undergoing root complex-focused procedures electively were stratified by valvulopathy (ie, aortic stenosis vs aortic insufficiency) and substratified into ascending or root aneurysm phenotype. Inverse probability weights were calculated to adjust for baseline differences. RESULTS: Kaplan-Meier curves for all-cause mortality demonstrated no difference between Bentall versus AVRSCAAR for aortic stenosis and aortic insufficiency presentations (log-rank P > .05). In patients with aortic stenosis, multivariable Cox regression showed significantly decreased risk of stroke for biologic AVRSCAAR (hazard ratio, 0.04; P = .013). Aortic reoperation rates were similar for biologic versus mechanical valves (P = .353). In patients with aortic insufficiency, similar long-term mortality (hazard ratio, 0.95; P = .93), but lower stroke risk in biologic AVRSCAAR group by Cox regression, and lower aortic reoperation rate was noted (coefficient < 0.01; P < .001). Comparing Bentall to VSRR, mortality (hazard ratio, 0.12; P = .022) was significantly improved in patients undergoing VSRR, but recurrence of moderate or greater aortic insufficiency was higher in VSRR by multistate model (beta coefficient 2.63; P < .001). CONCLUSIONS: A tailored approach to heterogeneous ascending aneurysm pathologies in bicuspid aortic valve syndrome utilizing Bentall, AVRSCAAR, and VSRR procedures renders excellent long-term clinical and functional outcomes, with biologic conduits showing equivalent to improved clinical outcomes.

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PURPOSE: Three-dimensional (3D) quantification of circulation using a Finite Elements methodology. METHODS: We validate our 3D method using an in-silico arch model, for different mesh resolutions, image resolution and noise levels, and we compared this with a currently used 2D method. Finally, we evaluated the application of our methodology in 4D Flow MRI data of ascending aorta of six healthy volunteers, and six bicuspid aortic valve (BAV) patients, three with right and three with left handed flow, at peak systole. The in-vivo data was compared using a Mann-Whitney U-test between volunteers and patients (right and left handed flow). RESULTS: The robustness of our method throughout different image resolutions and noise levels showed subestimation of circulation less than 45 cm2 /s in comparison with the 55cm2 /s generated by the current 2D method. The circulation (mean ± SD) of the healthy volunteer group was 13.83 ± 28.78 cm2 /s, in BAV patients with right-handed flow 724.37 ± 317.53 cm2 /s, and BAV patients with left-handed flow -480.99 ± 387.29 cm2 /s. There were significant differences between healthy volunteers and BAV patients groups (P-value < .01), and also between BAV patients with a right-handed or left-handed helical flow and healthy volunteers (P-value < .01). CONCLUSION: We propose a novel 3D formulation to estimate the circulation in the thoracic aorta, which can be used to assess the differences between normal and diseased hemodynamic from 4D-Flow MRI data. This method also can correctly differentiate between the visually seen right- and left-handed helical flow, which suggests that this approach may have high clinical sensitivity, but requires confirmation in longitudinal studies with a large cohort.

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OBJECTIVES: Bicuspid aortic valve (BAV) is an important aetiology of aortic stenosis and the use of transcatheter aortic valve implantation (TAVI) has not been fully explored in this cohort. This systematic review and meta-analysis compared the outcomes of TAVI in stenotic BAV against tricuspid aortic valve (TAV). METHODS: An electronic literature search was performed in PubMed, MEDLINE, EMBASE, and Scopus to identify all studies comparing TAVI in stenotic BAV versus TAV. Only studies comparing TAVI in BAV versus TAV were included, without any limit on the study date. Primary endpoints were 30-day and 1-year mortality, while secondary endpoints were postoperative rates of stroke, acute kidney injury (AKI), and permanent pacemaker (PPM) requirement. A trial sequential analysis (TSA) was performed for all endpoints to understand their significance. RESULTS: Thirteen studies met the inclusion criteria (917 BAV and 3079 TAV patients). The BAV cohort was younger (76.8±7.43 years vs. 78.5±7.12 years, P=0.02), had a higher trans-aortic valve gradient (P=0.02), and larger ascending aortic diameters (P<0.0001). No significant difference was shown for primary (30-day mortality [P=0.45] and 1-year mortality [P=0.41]) and secondary endpoints (postoperative stroke [P=0.49], AKI [P=0.14], and PPM requirement [P=0.86]). The BAV group had a higher rate of significant postoperative aortic regurgitation (P=0.002). TSA showed that there was sufficient evidence to conclude the lack of difference in PPM requirements, and 30-day and 1-year mortality between the two cohorts. CONCLUSION: TAVI gives satisfactory outcomes for treating stenotic BAV and should be considered clinically.

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The presence of a horizontal aorta in patients treated with transcatheter aortic valve replacement increases the difficulty of the procedure. We present 5 cases with aortic stenosis with a horizontal aorta who underwent transcatheter aortic valve replacement using a self-expanding prostheses, with the objective of describing the techniques used and obtaining success with a snare catheter. (Level of Difficulty: Intermediate.).

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BACKGROUND: There is a rising trend for transcatheter aortic valve implantation (TAVI) in bicuspid aortic stenosis patients. Data on the use of self-expandable (SEV) vs. balloon-expandable (BEV) valves in these patients are scarce. Therefore, we systematically compared clinical outcomes in bicuspid aortic stenosis patients treated with SEV and BEV. METHODS: Data were extracted from PubMed/MEDLINE, EMBASE, CENTRAL/CCTR, ClinicalTrials.gov, SciELO, LILACS, Google Scholar and reference lists of relevant articles. Eight studies published from 2013 to 2020 including a total of 1,080 patients (BEV: n = 620; SEV: n = 460) were selected. Primary endpoints were procedural, 30-day and 1-year mortality. Secondary endpoints were new pacemaker implantation, annular rupture, coronary obstruction, moderate-to-severe paravalvular leak, need of second valve, stroke and acute kidney injury. RESULTS: We found no statistically significant difference in mortality between patients treated with BEV vs. SEV during index procedure, at 30 days and at 1 year. BEVs showed a statistically significant higher risk of annulus rupture (2.5%) in comparison with SEV (0%) (OR 5.81 [95% CI, 3.78-8.92], p < .001). New generation BEVs were also associated with significantly less paravalvular leak when compared to new generation SEVs (OR 0.08 [95% CI, 0.02-0.35], p = .001). CONCLUSIONS: This meta-analysis of observational studies of TAVI for bicuspid valves, showed no difference in short- and mid-term TAVI mortality with BEVs and SEVs. BEVs presented a higher risk of annular rupture in comparison with SEV.

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