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ObjectiveWomen with Turner syndrome (TS) are at increased risk of aortic dissection, which is a life-threatening event associated with aortic dilation. Knowledge on the development of aortic dilation over time remains limited. This study aims to describe the prevalence of aortic dilation, to find associated factors and to study aortic growth in women with TS.MethodsIn this prospective multicentre cohort study, consecutive adult women with genetically proven TS included between 2014 and 2016 underwent ECG-triggered multiphase CT angiography at baseline and after 3 years. Aortic diameters were measured at seven levels of the thoracic aorta using double oblique reconstruction and indexed for body surface area. Ascending aortic dilation was defined as an aortic size index >20 mm/m2. Aorta-related and cardiovascular events were collected. Statistical analysis included linear and logistic regression and linear mixed effects models.ResultsThe cohort consisted of 89 women with a median age of 34 years (IQR: 24–44). Ascending aortic dilation was found in 38.2% at baseline. At baseline, age (OR: 1.08 (95% CI 1.03 to 1.13), p<0.001), presence of bicuspid aortic valve (BAV) (OR: 7.09 (95% CI 2.22 to 25.9), p=0.002) and systolic blood pressure (OR: 1.06 (95% CI 1.02 to 1.11), p=0.004) were independently associated with ascending aortic dilation. During a median follow-up of 3.0 (2.4–3.6) years (n=77), significant aortic growth was found only at the sinotubular junction (0.20±1.92 mm, p=0.021). No aortic dissection occurred, one patient underwent aortic surgery and one woman died.ConclusionsIn women with TS, ascending aortic dilation is common and associated with age, BAV and systolic blood pressure. Aortic diameters were stable during a 3-year follow-up, apart from a significant yet not clinically relevant increase at the sinotubular junction, which may suggest a more benign course of progression than previously reported.

ObjectiveBicuspid aortic valve (BAV) is the most common congenital heart disease. This study aimed to determine the prevalence rate of BAV in first-degree relatives (FDR) and the inheritance pattern according to different morphotypes and aortic dilation.MethodsBAV probands were consecutively studied at eight tertiary referral centres. After sequential screening, FDR were included in the study. The BAV morphotype, aortic dilation and aortic phenotype were assessed by transthoracic echocardiography.ResultsSeven hundred and twenty-four FDR of 256 BAV probands agreed to undergo family screening. The prevalence of BAV was 6.4% in FDR (9.2% in men, 3.5% in women, p=0.002). Aortic dilation was diagnosed in 9.6% of FRD with tricuspid aortic valves (TAV), with a root phenotype in 2.7% and tubular in 6.9% and more frequently in the presence of arterial hypertension (OR 4.48; CI 95% 2.51 to 7.99; p=0.0001) and valvular regurgitation (OR 5.87, CI 95% 1.37 to 25.16; p=0.025). The heritability (h2 ) of BAV was highly significant (0.47; p=0.002); however, no concordance was observed among valve morphotypes. Aortic dilation heritability was not significant.ConclusionsThe BAV prevalence rate in FDR was low (6.4%) but aortic dilation was observed in 9.6% of FDR with TAV. The heritability of BAV was high without concordance in valve morphotypes, and aortic dilation heritability was not observed. Patients with BAV should be made aware of its familial pattern.

•Risk of maternal complications during pregnancy with bicuspid aortic valve is incompletely understood.•In 20 years of pregnancies in women with bicuspid aortic valve, no major adverse events occurred.•Most patients had a single transthoracic echocardiogram to assess the aortic valve and thoracic aorta.•Single transthoracic echocardiogram is likely sufficient if no high-risk features are identified.

Aortic wall shear stress (WSS) is a known predictor of ascending aortic growth in patients with a bicuspid aortic valve (BAV). The aim of this study was to study regional WSS and changes over time in BAV patients. BAV patients and age-matched healthy controls underwent four-dimensional (4D) flow cardiovascular magnetic resonance (CMR). Regional, peak systolic ascending aortic WSS, aortic valve function, aortic stiffness measures, and aortic dimensions were assessed. In BAV patients, 4D flow CMR was repeated after 3 years of follow-up and both at baseline and follow-up computed tomography angiography (CTA) were acquired. Aortic growth (volume increase of ≥5%) was measured on CTA. Regional WSS differences within patients’ aorta and WSS changes over time were analyzed using linear mixed-effect models and were associated with clinical parameters. Thirty BAV patients (aged 34 years [interquartile range (IQR) 25–41]) were included in the follow-up analysis. Additionally, another 16 BAV patients and 32 healthy controls (aged 33 years [IQR 28–48]) were included for other regional analyses. Magnitude, axial, and circumferential WSS increased over time (all p < 0.001) irrespective of aortic growth. The percentage of regions exposed to a magnitude WSS >95th percentile of healthy controls increased from 21% (baseline 506/2400 regions) to 31% (follow-up 734/2400 regions) (p < 0.001). WSS angle, a measure of helicity near the aortic wall, decreased during follow-up. Magnitude WSS changes over time were associated with systolic blood pressure, peak aortic valve velocity, aortic valve regurgitation fraction, aortic stiffness indexes, and normalized flow displacement (all p < 0.05). An increase in regional WSS over time was observed in BAV patients, irrespective of aortic growth. The increasing WSSs, comprising a larger area of the aorta, warrant further research to investigate the possible predictive value for aortic dissection. [Display omitted]

The clinical and imaging differences between bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) patients with medically managed asymptomatic moderate-to-severe aortic stenosis (AS) have not been studied previously. We aim to characterize these differences and their clinical outcomes in this study. A retrospective observational study was conducted on 836 consecutive cases of isolated asymptomatic moderate-to-severe AS, with median follow-up of 3.4 years. Clinical and echocardiographic characteristics were compared between BAV and TAV patients. Subgroup analysis stratified by AS severity were performed. Survival analysis of all-cause mortality was performed using Kaplan-Meier curves and Cox proportional hazards model. Compared to BAV patients, TAV patients were older (76 ± 11 vs 55 ± 16 years, p <0.001) and had more co-morbidities including hypertension (78% vs 56%; p <0.001), diabetes (41% vs 24%; p <0.001), and chronic kidney disease (20% vs 3%; p = 0.001). TAV patients had less severe aortic valve disease than BAV patients, with a higher aortic valve area index (0.71 ± 0.20 cm2/m2 vs 0.61 ± 0.18 cm2/m2, p <0.001) and less aortic dilation (sinotubular junction: 23.7 ± 4.0 mm vs 26.9 ± 4.8 mm, p <0.001; mid-ascending aorta: 31.4 ± 4.7 mm vs 36.3 ± 6.3 mm, p <0.001). TAV patients were more likely to have eccentric left ventricular hypertrophy and less likely to have a normal geometry (p = 0.003). Competing risk analysis identified increased age (hazard ratio 1.03, 95% confidence interval 1.02 to 1.05, p <0.001) and LVEF (hazard ratio 0.98, 95% confidence interval 0.97 to 0.99, p <0.001) as independent risk factors of all-cause mortality. Valve morphology was not a significant independent risk factor for aortic valve replacement or mortality. In conclusion, asymptomatic TAV patients had more cardiovascular risk factors, less severe aortic valve disease, less sinotubular and mid-ascending aortic dilation, more severe LV remodeling.

Aortic dilation is seen in pediatric/young adult patients with bicuspid aortic valve (BAV), and hemodynamic markers to predict aortic dilation are necessary for monitoring. Although promising hemodynamic metrics, such as abnormal wall shear stress (WSS) magnitude, have been proposed for adult BAV patients using four-dimensional (4D) flow cardiovascular magnetic resonance, those for pediatric BAV patients have less frequently been reported, partly due to scarcity of data to define normal WSS range. To circumvent this challenge, this study aims to investigate if a recently proposed 4D flow-based hemodynamic measurement, abnormal flow directionality, is associated with aortic dilation in pediatric/young adult BAV patients. 4D flow scans for BAV patients (<20 years old) and age-matched controls were retrospectively enrolled. Static segmentation for the aorta and pulmonary arteries was obtained to quantify peak systolic hemodynamics and diameters in the proximal aorta. In addition to peak velocity, WSS, vorticity, helicity, and viscous energy loss, direction of aortic velocity and WSS in BAV patients were compared with that of control atlas using registration technique; angle differences of >60 deg and >120 deg were defined as moderately and severely abnormal, respectively. The association between the obtained metrics and normalized diameters (Z-scores) was evaluated at the sinotubular junction, mid-ascending aorta, and distal ascending aorta. Fifty-three BAV patients, including 18 with history of repaired aortic coarctation, and 17 controls were enrolled. Correlation between moderately abnormal velocity/WSS direction and aortic Z-scores was moderate to strong at the sinotubular junction and mid-ascending aorta (R = 0.62–0.81; p < 0.001) while conventional measurements exhibited weaker correlation (|R| = 0.003–0.47, p = 0.009–0.99) in all subdomains. Multivariable regression analysis found moderately abnormal velocity direction and existence of aortic regurgitation (only for isolated BAV group) were independently associated with mid-ascending aortic Z-scores. Abnormal velocity and WSS directionality in the proximal aorta were strongly associated with aortic Z-scores in pediatric/young adult BAV patients. [Display omitted]

Background Abnormal blood flow patterns are known to contribute to the ascending aortic dilation in patients with bicuspid aortic valve (BAV). The present study elucidated the blood flow characteristics in the dilated ascending aorta before and after transcatheter aortic valve replacement (TAVR) using computational fluid dynamics (CFD) analysis. Methods We performed CFD analysis in three BAV patients with ascending aortic dilation (maximum diameter ≥ 45 mm) who underwent TAVR. The blood flow streamline was visualized to evaluate the pre- and post-operative flow velocity, severity of vortex and helix, and wall shear stress (WSS) in the ascending aorta. Results Before the procedure, all three patients showed abnormal blood flow patterns, with vortex and helix in the ascending aorta. Regionally elevated WSS was also observed in the lateral or posterior ascending aortic wall (16.7 Pa, 12.2 Pa, and 14.5 Pa in patient 1, 2, and 3, respectively). After the procedure, the blood flow patterns significantly improved, and the maximum WSS also decreased (4.2 Pa, 1.1 Pa, and 3.2 Pa in patient 1, 2, and 3, respectively). Conclusion Abnormal blood flow patterns and WSS appeared to improve after TAVR in BAV patients with ascending aortic dilation. The impact on the long-term aortic growth rate and the incidence of aortic dissection requires further studies. Trial Registration Changes of Ascending Aortic Diameter in Patients Undergoing Transcatheter Aortic Valve Replacement. ClinicalTrial.gov number NCT05739253. Trial registration date 20,230,212.

Atherosclerotic plaques impair vascular function and can lead to arterial obstruction and tissue ischaemia. Rupture of an atherosclerotic plaque within a coronary artery can result in an acute myocardial infarction, which is responsible for significant morbidity and mortality worldwide. Prompt reperfusion can salvage some of the ischaemic territory, but ischaemia and reperfusion (IR) still causes substantial injury and is, therefore, a therapeutic target for further infarct limitation. Numerous cardioprotective strategies have been identified that can limit IR injury in animal models, but none have yet been translated effectively to patients. This disconnect prompts an urgent re-examination of the experimental models used to study IR. Since coronary atherosclerosis is the most prevalent morbidity in this patient population, and impairs coronary vessel function, it is potentially a major confounder in cardioprotective studies. Surprisingly, most studies suggest that atherosclerosis does not have a major impact on cardioprotection in mouse models. However, a major limitation of atherosclerotic animal models is that the plaques usually manifest in the aorta and proximal great vessels, and rarely in the coronary vessels. In this review, we examine the commonly used mouse models of atherosclerosis and their effect on coronary artery function and infarct size. We conclude that none of the commonly used strains of mice are ideal for this purpose; however, more recently developed mouse models of atherosclerosis fulfil the requirement for coronary artery lesions, plaque rupture and lipoprotein patterns resembling the human profile, and may enable the identification of therapeutic interventions more applicable in the clinical setting.

Our study was to apply the 2015 American Heart Association/American College of Cardiology Athletic Participation Guidelines to a group of otherwise healthy school age children and young adults with bicuspid aortic valve (BAV) and describe the potential competitive sports restriction as they age. We performed a retrospective chart review of children and young adults aged 5 to 22 years with isolated BAV with at least two echocardiograms between 2000 and 2013. Using task force guidelines, exercise restriction was recommended for any of the following: (1) any dilation of the aortic root, (2) any dilation of the ascending aorta, (3) moderate aortic stenosis, (4) severe aortic regurgitation; (5) left ventricular dilation or (6) reduced shortening fraction. Of the 345 patients with isolated BAV, 202 were considered restricted at study entry. The final cohort included 123 children and young adults. Over the course of follow up, 36% (44 of 123) met restriction criteria. The most likely cause for restriction was aortic dilation (34%). Progression of aortic valve disease occurred in a minority of patients (3%). There were no reports of death, dissection or catheter or surgical based intervention. In conclusion, we found that strict adherence to current guidelines would result in restriction of more than 1/3 of school age children and young adults with BAV from some form of competitive athletics during school age years. Strict application of the current guidelines in this age group may lead to over-restriction of youths from competitive sports.

In an era of rapidly expanding use of transcatheter aortic valve implantation (TAVI), the management of patients with bicuspid aortic valve (BAV) disease is far less well established than in those with trileaflet anatomy. Results of isolated surgical aortic valve replacement are excellent in suitable patients, and surgery also allows treatment of concomitant pathology of the aortic root and ascending aorta that is frequently encountered in this cohort. Conversely, TAVI provides an excellent alternative in older patients who may be unsuitable for surgery, although outcomes in BAV disease have only been reported in relatively small non-randomised series. Here, we discuss the pertinent literature on this topic and outline contemporary interventional treatment options in this challenging setting.