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ABSTRACT: Background: 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. Methods: 4D flow scans for BAV patients (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. Results: 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

Background: The measurement of aortic dimensions and their evolution are key in the management of patients with aortic diseases. Manual assessment, the current guideline-recommended method and clinical standard, is subjective, poorly reproducible, and time-consuming, limiting the capacity to track aortic growth in everyday practice. Aortic geometry mapping (AGM) via image registration of serial computed tomography angiograms outperforms manual assessment, providing accurate and reproducible 3D maps of aortic diameter and growth rate. This observational study aimed to evaluate the accuracy and reproducibility of AGM on non-gated contrast-enhanced (CE-) and cardiac- and respiratory-gated (GN-) magnetic resonance angiographies (MRA). Methods: Patients with thoracic aortic disease followed with serial CE-MRA (n = 30) or GN-MRA (n = 15) acquired at least 1 year apart were retrospectively and consecutively identified. Two independent observers measured aortic diameters and growth rates (GR) manually at several thoracic aorta reference levels and with AGM. Agreement between manual and AGM measurements and their inter-observer reproducibility were compared. Reproducibility for aortic diameter and GR maps assessed with AGM was obtained. Results: Mean follow-up was 3.8 ± 2.3 years for CE- and 2.7 ± 1.6 years for GN-MRA. AGM was feasible in the 93% of CE-MRA pairs and in the 100% of GN-MRA pairs. Manual and AGM diameters showed excellent agreement and inter-observer reproducibility (ICC>0.9) at all anatomical levels. Agreement between manual and AGM GR was more limited, both in the aortic root by GN-MRA (ICC=0.47) and in the thoracic aorta, where higher accuracy was obtained with GN- than with CE-MRA (ICC=0.55 vs 0.43). The inter-observer reproducibility of GR by AGM was superior compared to manual assessment, both with CE- (thoracic: ICC= 0.91 vs 0.51) and GN-MRA (root: ICC=0.84 vs 0.52; thoracic: ICC=0.93 vs 0.60). AGM-based 3D aortic size and growth maps were highly reproducible (median ICC >0.9 for diameters and >0.80 for GR). Conclusion: Mapping aortic diameter and growth on MRA via 3D image registration is feasible, accurate and outperforms the current manual clinical standard. This technique could broaden the possibilities of clinical and research evaluation of patients with aortic thoracic diseases.

Abstract Background Coarctation of the aorta (COA) is associated with reduced aortic distensibility and systemic hypertension (HTN). 60–85% of COA patients have a bicuspid aortic valve (BAV). It is not known if the presence of a BAV accentuates the aortopathy and HTN in CoA patients. We examined whether patients with COA and a BAV had lower aortic distensibility by CMR, and a higher prevalence of systemic HTN compared with COA patients with a tricuspid aortic valve (TAV). Methods In successfully repaired COA patients excluding those with residual COA, ascending aorta (AAO) and descending aorta (DAO) distensibility was calculated by CMR. HTN was assessed using standard pediatric and adult criteria. Results Among 215 COA patients (median age 25.3 years), 67% had a BAV, and 33% had a TAV. Median AAO distensibility z-score was lower in the BAV group (− 1.2 versus − 0.7; p = 0.014) but DAO distensibility was similar in BAV and TAV patients. HTN prevalence was similar in BAV (32%) and TAV groups (36%, p = 0.56). On multivariable analysis controlling for confounders, HTN was not associated with BAV but was associated with male gender (p = 0.003) and older age at follow-up (p = 0.004). Conclusions In young adults with treated COA, those with a BAV had stiffer AAO compared to those with a TAV, but DAO stiffness was similar. HTN was not related to BAV. These results suggest that although the presence of a BAV in COA exacerbates the AAO aortopathy, it does not exacerbate the more generalized vascular dysfunction and associated HTN.

Abstract Background Bicuspid aortic valve (BAV) disease is associated with increased risk of aortopathy. In addition to current intervention guidelines, BAV mediated changes in aortic 3D hemodynamics have been considered as risk stratification measures. We aimed to evaluate the association of 4D flow cardiovascular magnetic resonance (CMR) derived voxel-wise aortic reverse flow with aortic dilation and to investigate the role of aortic valve regurgitation (AR) and stenosis (AS) on reverse flow in systole and diastole. Methods 510 patients with BAV (52 ± 14 years) and 120 patients with trileaflet aortic valve (TAV) (61 ± 11 years) and mid-ascending aorta diameter (MAAD) > 35 mm who underwent CMR including 4D flow CMR were retrospectively included. An age and sex-matched healthy control cohort (n = 25, 49 ± 12 years) was selected. Voxel-wise reverse flow was calculated in the aorta and quantified by the mean reverse flow in the ascending aorta (AAo) during systole and diastole. Results BAV patients without AS and AR demonstrated significantly increased systolic and diastolic reverse flow (222% and 13% increases respectively, p 17% increase, p 27% decrease, p 0.1). Conclusion 4D flow CMR derived reverse flow associated with BAV was successfully captured even in the absence of AR or AS and in comparison to TAV patients with aortic dilation. Diastolic AAo reverse flow increased with AR severity while AS severity strongly correlated with increased systolic reverse flow in the AAo. Additionally, increasing MAAD was independently associated with increasing systolic AAo reverse flow. Thus, systolic AAo reverse flow may be a valuable metric for evaluating disease severity in future longitudinal outcome studies.

Background: 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., Methods: 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., Results: 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., Conclusion: Abnormal velocity and WSS directionality in the proximal aorta were strongly associated with aortic Z-scores in pediatric/young adult BAV patients., Competing Interests: Declaration of competing interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: The measurement of aortic dimensions and their evolution are key in the management of patients with aortic diseases. Manual assessment, the current guideline-recommended method and clinical standard, is subjective, poorly reproducible, and time-consuming, limiting the capacity to track aortic growth in everyday practice. Aortic geometry mapping (AGM) via image registration of serial computed tomography angiograms outperforms manual assessment, providing accurate and reproducible 3D maps of aortic diameter and growth rate. This observational study aimed to evaluate the accuracy and reproducibility of AGM on non-gated contrast-enhanced (CE-) and cardiac- and respiratory-gated (GN-) magnetic resonance angiographies (MRA)., Methods: Patients with thoracic aortic disease followed with serial CE-MRA (n = 30) or GN-MRA (n = 15) acquired at least 1 year apart were retrospectively and consecutively identified. Two independent observers measured aortic diameters and growth rates (GR) manually at several thoracic aorta reference levels and with AGM. Agreement between manual and AGM measurements and their inter-observer reproducibility were compared. Reproducibility for aortic diameter and GR maps assessed with AGM was obtained., Results: Mean follow-up was 3.8 ± 2.3 years for CE- and 2.7 ± 1.6 years for GN-MRA. AGM was feasible in the 93% of CE-MRA pairs and in the 100% of GN-MRA pairs. Manual and AGM diameters showed excellent agreement and inter-observer reproducibility (ICC>0.9) at all anatomical levels. Agreement between manual and AGM GR was more limited, both in the aortic root by GN-MRA (ICC=0.47) and in the thoracic aorta, where higher accuracy was obtained with GN- than with CE-MRA (ICC=0.55 vs 0.43). The inter-observer reproducibility of GR by AGM was superior compared to manual assessment, both with CE- (thoracic: ICC= 0.91 vs 0.51) and GN-MRA (root: ICC=0.84 vs 0.52; thoracic: ICC=0.93 vs 0.60). AGM-based 3D aortic size and growth maps were highly reproducible (median ICC >0.9 for diameters and >0.80 for GR)., Conclusion: Mapping aortic diameter and growth on MRA via 3D image registration is feasible, accurate and outperforms the current manual clinical standard. This technique could broaden the possibilities of clinical and research evaluation of patients with aortic thoracic diseases., Competing Interests: Declaration of Competing Interest The authors declare that they have no competing interests., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Quadricuspid aortic valve (QAV) is a rare congenital heart disease with a limited body of literature. This retrospective cohort study investigates QAV morphology, function, and clinical outcomes., Methods: Echocardiography was used to assess valvular function. Morphological characteristics such as phenotypes, raphe, regurgitant orifice area (ROA), and aortic dilation (diameter >40 ​mm) were assessed by cardiac CT. Patients were followed up for the combined event of all-cause death and aortic valve replacement (AVR)., Results: Ninety QAV patients (screened from 322385 CT scans) were included (mean age 55.2 ​± ​13.6 years, 61.1 ​% male). Isolated significant aortic regurgitation (AR) was present in 75.6 ​% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 ​%) and type II (3 larger and 1 smaller leaflets, 42.2 ​%) QAV. Fused raphe was present in 26.7 ​% of patients. ROA CT was correlated with AR severity and aortic dilation (41.1 ​%, n ​= ​37). Among patients without AVR at baseline (n ​= ​60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3-62.8). ROA CT was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 ​mm 2 , HR ​= ​4.25, 95%CI 1.49-12.17, p ​= ​0.007; as a continuous variable (per mm 2 increment), HR ​= ​1.04, 95%CI 1.01-1.07, p ​= ​0.003). Additionally, ROA CT had incremental prognostic value when added to the AR severity model (area under the receiver-operating characteristic curve increased from 86.8 to 88.4, p ​= ​0.004)., Conclusion: QAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROA CT may be a potential ancillary prognostic marker in patients with QAV., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Aortic stenosis is one of the most prevalent cardiac valvular diseases throughout the world and has a significant impact on quality of life. While there are several etiologies, we will be discussing the case of a male in his mid-thirties of southeast Asian descent with a bicuspid aortic valve which was found to be functionally unicuspid and complicated by aortic dilation. Following a comprehensive review of literature, it appears this subset of aortic stenosis is not commonly encountered. In addition to presenting this fascinating case, we will review the epidemiology, classification and management of aortic stenosis. Furthermore, we will examine the latest evidence-based literature on bicuspid aortic valve and unicuspid aortic valve and discuss interventions and diagnostic tools that may improve clinical prognosis., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023. Published by Elsevier Inc.)

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., Competing Interests: Funding Support and Author Disclosures Funding was provided by National Institutes of Health (grant nos. R01HL115828, R01HL133504, and F30HL145995). Dr Soulat received a grant support from the French College of Radiology Teachers and French Radiology Society. Additional support was provided by the Melman Bicuspid Aortic Valve Program, Bluhm Cardiovascular Institute. Dr Malaisrie has received honoraria and a research grant from Terumo Aortic. Dr McCarthy has received royalties and honoraria for speaking for Edwards Lifesciences. Dr Markl has received research support from Siemens Healthineers; a research grant and consulting fees from Circle Cardiovascular Imaging; and a research grant from Cryolife Inc. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Purpose: Cardiac-valvular and vascular Ehlers-Danlos syndrome (EDS) have significant cardiovascular issues. The prevalence and significance of such abnormalities in classical (cEDS) or hypermobile EDS (hEDS) remain unclear. We report the prevalence of cardiac abnormalities in patients with cEDS and hEDS., Methods: We identified 532 pediatric patients with potential EDS evaluated at our institution from January 2014 through April 2019 by retrospective chart review. Ninety-five patients (12 cEDS and 83 hEDS patients) met 2017 EDS diagnostic criteria and had an echocardiogram. One patient was excluded due to complex congenital heart disease, and two were excluded due to lack of images. We reviewed echocardiograms for all structural abnormalities., Results: Of these 95 patients, 1 had mild aortic root dilation, and 1 had mild ascending aorta dilation in the setting of a bicuspid aortic valve. Eleven patients (11.6%) had a cardiac valve abnormality, all of which were trivial to mild. None of the patients required cardiac intervention., Conclusion: Our results demonstrate that aortic dilation and valvular anomalies are uncommon in cEDS or hEDS patients. Given the lack of evidence, we do not recommend echocardiographic evaluation and surveillance in patients with cEDS and hEDS in the absence of clinical findings or positive family history.

Objectives: Large-vessel involvement (LVI) can occur in giant-cell arteritis (GCA) and may represent a distinct disease subgroup with a higher risk for aortic dilation. This study aimed to better characterize the presentation and evolution of LVI in patients with GCA., Patients and Methods: A retrospective multicenter study enrolled 248 GCA patients with LVI and 301 GCA patients without LVI on imaging. Factors associated with aortic dilation were identified in a multivariable model., Results: The patients with LVI were younger (p<0.0001), more likely to be women (p=0.01), and showed fewer cephalic symptoms (p<0.0001) and polymyalgia rheumatica (p=0.001) but more extracranial vascular symptoms (p=0.05) than the patients without LVI. Glucocorticoids (GC) management did not differ between the two groups, but the GC discontinuation rate was lower in the patients with LVI (p=0.0003). Repeated aortic imaging procedures were performed at 19months [range: 5-162months] and 17months [range: 6-168months] after diagnosis in 154 patients with LVI and 123 patients without LVI, respectively, of whom 21% and 7%, respectively, presented new aortic dilations (p=0.0008). In the patients with LVI, aortic dilation occurred on an aorta segment shown to be inflammatory on previous imaging in 94% of patients. In the multivariate analysis, LVI was the strongest predictor of aortic dilation (hazard ratio: 3.16 [range: 1.34-7.48], p=0.009)., Conclusions: LVI represents a distinct disease pattern of GCA with an increased risk of aortic dilation. Control of the aortic morphology during follow-up is required., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: The present study assessed whether descending thoracic aorta growth can be measured reliably by volumetric analysis using multi-detector row computed tomography (MDCT) and whether growth influences the need for future aortic interventions in survivors of acute type A aortic dissection., Methods: A total of 51 patients (58 ± 11 years, 61% male) who underwent surgery for type A aortic dissection with ≥2 postoperative MDCT scans ≥5 months apart were included. Volumetric analysis of the descending thoracic aorta was performed with acceptable intraobserver variability. Growth of the complete, false and true lumen was estimated in ml/year and defined as slow growth (≤average growth) or fast growth (>average growth)., Results: The complete lumen volume increased from 133 ± 8 ml to 163 ± 9 ml after 3.5 years follow-up (p < 0.001), with an average growth rate of 6.1 ml/year. The false lumen volume increased from 81 ± 7 ml to 106 ± 12 ml (p = 0.018) with an average growth rate of 2.8 ml/year. The true lumen changed only slightly from 59 ± 4 ml to 65 ± 8 ml (p = 0.205). Five-year freedom from descending thoracic aorta intervention was significantly lower in patients with above-average growth of the complete lumen (80 ± 9%) compared to slow growth (100%; p = 0.003). Similar observations were made for the false lumen (fast: 74 ± 12% vs. slow: 100%; p = 0.042)., Conclusions: Increased growth of the false lumen of the descending thoracic aorta after type A aortic dissection was associated with a higher risk of secondary interventions., (Copyright © 2016 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Background: Aortic dilation and dissection contribute highly to the increased mortality of Turner syndrome (TS) but the exact pathophysiology is not completely understood., Design: Prospective case - control study., Methods: 15 prepubertal TS girls (median age 10.64, IQ 8.31-11.04) with a tricuspid (TAV, n=9) or a bicuspid (BAV, n=6) aortic valve, and 31 sex-, age-, and height-matched healthy controls underwent a cardiac and vascular ultrasound to evaluate aortic dimensions and elastic properties of the aortic wall., Results: TS BAV had significantly larger ascending aortic diameters than controls for absolute diameter, 22.2±5.1mm vs. 18.6±1.9mm (p=0.014) and z-score 1.7±2.1 vs. 0.1±0.7 (p=0.008). Distensibility of the ascending aorta was lower in the TS than in controls (40.2×10 -3 kPa -1 , IQ 31.3-56.2 vs. 62.9×10 -3 kPa -1 , IQ 55.5-76.5, p=0.003), both for TS TAV (p=0.014) and BAV (p=0.005). Stiffness index was higher in TS than in controls (5.26, IQ 3.34-5.26 vs. 3.23, IQ 2.55-3.24, p=0.005), both for TS TAV (p=0.028) and TS BAV (p=0.006). Pulse wave velocity was not different between groups. There was no correlation between stiffness and z-score of the ascending aortic diameter., Conclusions: In prepubertal TS girls, stiffness of the ascending aorta is increased in patients with a BAV and TAV while dilation of the ascending aorta is more frequent in BAV. This suggests an intrinsic aortic wall abnormality making all TS patients at increased risk for severe aortic complications although the risk is the highest for TS with BAV., (Copyright © 2016 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Background/objectives: The mechanisms underlying aortic dilation in bicuspid aortic valve (BAV) disease are unknown. Circulating endothelial microparticles (EMPs) have emerged as biomarkers of endothelial damage. We sought to evaluate the relationships among EMPs, BAV disease, and aortic dilation., Methods: Four evaluations were used. Circulating EMPs (PECAM(+), E-selectin(+)) were compared between BAV patients and tricuspid aortic valve (TAV) control subjects. The variables related to circulating EMPs were investigated in BAV patients. Circulating EMP levels were compared between BAV and TAV patients with a dilated aorta. Finally, circulating EMPs in BAV patients were evaluated over time with respect to aortic valve surgery (AVS) or aortic surgery., Results: We observed higher levels of circulating PECAM(+) EMPs in the BAV patients than in the control subjects (3.98±0.2 vs. 2.39±0.4 per log PECAM(+) EMPs/μl, p=0.001). Aortic dilation was the most significant variable that correlated with the PECAM(+) EMP levels in the BAV patients (β=0.321, p=0.008). The BAV patients with aortic dilation exhibited higher PECAM(+)EMP levels than the TAV patients with dilated aortas, and this correlation was independent of aortic valve function. We observed a drastic decrease in the circulating PECAM(+) EMPs following AVS and aortic root replacement (4.27±0.6 and 1.75±0.3 per log PECAM(+)EMPs/μl, p=0.002)., Conclusion: The observed pattern of higher circulating PECAM(+) EMP levels links BAV disease to endothelial damage and aortic dilation. Circulating PECAM(+) EMPs were identified as a biological variable related to aortic dilation in patients with BAV disease., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Sudden cardiovascular deaths in athletes are rare and only a fraction are due to aortic events. There has been concern that the hemodynamic load during exercise may lead to aortic dilation, but aortic dimensions in endurance and strength-trained athletes are only slightly larger than those in sedentary comparison subjects. The presence of a bicuspid aortic valve without significant valvular dysfunction and normal aortic dimensions should not influence eligibility to practice sport. Patients with genetic syndromes associated with aortopathy generally should be restricted from vigorous sports participation. This article reviews the diagnosis and management of diseases of the aorta in athletes., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objectives: This study sought to identify risk factors for rapid growth of the ascending aorta in patients with bicuspid aortic valve (BAV) disease, taking into account its phenotypic variability., Background: Phenotypic heterogeneity of BAV-related aortopathy has recently been widely recognized. However, few studies have addressed the determinants of aortic growth so far, not distinguishing among morphological phenotypes., Methods: Serial retrospective data on 133 adult outpatients with BAV undergoing echocardiographic follow-up were analyzed to search for factors associated with aortic diameter growth over time and with rapid aortic growth (fifth quintile of growth rate distribution), focusing on the impact of different valve morphotypes (i.e., cusp fusion pattern: right-left coronary [RL] and right-noncoronary [RN]) and previously defined aortic phenotypes (nondilated aorta, ascending dilation, root dilation)., Results: The RL pattern was present in 69% of patients with BAV and RN in 31%. At baseline, an ascending dilation phenotype was observed in 57% of patients and a root phenotype in 13.5%. No patient with RN-BAV had a root dilation phenotype at either baseline or last examination. Follow-up time averaged 4.0 ± 2.7 years (535 patient-years). The mean growth rate was 0.3 mm/year at the sinuses and 0.6 mm/year at the ascending level. Aortic regurgitation predicted an increase in ascending diameter over time (odds ratio [OR]: 2.3; p = 0.03). Root phenotype at presentation, not absolute baseline diameter, was an independent predictor of fast progression (>0.9 mm/year) for the ascending tract (OR: 14; p = 0.001). Fast growth was rarely seen in patients with the RL morphotype and ascending phenotype (6% at the root and 10% at the ascending level)., Conclusions: In patients with BAV, the root phenotype (aortic dilation predominantly at the sinuses, with normal or less dilated ascending tract) may be a marker of more severe aortopathy, warranting closer surveillance and earlier treatment. The more common ascending phenotype proved to be a more stable disease entity, generally with slower progression., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study evaluated predictors for aortic dilation (AD) in patients with repaired tetralogy of Fallot (rTOF) using magnetic resonance angiography (MRA)., Background: AD is common in patients with rTOF and may result in increased morbidity and mortality. There are no guidelines for evaluation of AD for rTOF patients., Methods: All adults with rTOF who previously underwent MRA had retrospective aortic measurements at the sinuses of Valsalva (SoV) and ascending aorta (AsAo). Rate of change in diameter was determined in patients with multiple MRAs. Chart review identified risk factors for AD. Univariate and multivariate analyses tested predictors of AD., Results: Of the 87 patients who met the inclusion criteria, 12 (14%) had AD. At baseline, mean diameter was 3.6 ± 0.6 cm and 3.1 ± 0.6 cm at the SoV and AsAo, respectively. The AsAo was larger than the SoV in 17%. Predictors of AD included male gender, age, right aortic arch, pregnancy, older age at complete repair, smoking, and systemic hypertension. Serial studies were available in 55 patients; the rate of growth was slow: 0.4 ± 0.9 mm/year (SoV) and 0.1 ± 0.8mm/year (AsAo)., Conclusions: AD is common in rTOF at the SoV and AsAo. Transthoracic echocardiography, which does not always image the AsAo as well as MRA, may not image AD in rTOF in cases in which the AsAo is dilated. Although several risk factors correlate with AD in rTOF, the rate of aortic growth is slow, suggesting that rTOF patients may not require frequent aortic imaging., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: A bicuspid aortic valve (BAV) is a common congenital heart disease, which affects 1-2% of the population. However, the relationship between BAVs and aortic dilation has not been sufficiently elucidated., Methods: A total of 241 BAV patients who were referred to this hospital for cardiac surgery over a 4.75-year period were included in this study. In addition to the clinical characteristics of the included patients, the morphological features of the aortic valve and aorta, the length of the left main coronary artery, and the laboratory findings (the coagulation and hematological parameters as well as the total cholesterol concentration) were determined and compared with those of the tricuspid aortic valve (TAV) patients., Results: The BAV patients were younger than the TAV patients for a valve surgery in the last 3 months of the study period. The BAV patients were predominantly male. Most of the BAVs that were surgically treated were stenotic, regurgitant, or combined, and only 19 (7.88%) were normally functioning valves. According to echocardiography or operative records, 148 (78.31%) were type A, 31 (16.40%) were type B, and 10 (5.29%) were type C. The left main coronary artery was much shorter in the BAV patients than it was in the TAV patients. There was no significant difference between BAV and TAV patients in the total cholesterol concentrations; whereas differences were noted between patients receiving lipid-lowering therapy and those not receiving lipid-lowering therapy. The dimensions of the aortic root, sinotubular junction, and ascending aorta were beyond normal limits, while they were significantly smaller in the BAV patients than in the TAV patients. They were also much smaller in patients receiving statin therapy than those not receiving statin therapy in both groups. Moreover, the aortic dilation in the BAV group was found to be significantly associated with patient age., Conclusions: The BAV patients developed aortic wall and aortic valve disorders at a younger age than the TAV patients and were predominantly male. Aortic dilation was observed in the aortic root, sinotubular junction, and ascending aortic segments in both the BAV and TAV patients undergoing surgical aortic valve replacement, although the BAV patients had a smaller degree of dilation than the TAV patients, and dilation was also significantly age-related in this group. The shorter left main coronary artery that the BAV patients possess may contribute to the progressive course of aortic dilation that these patients experience. Statin therapy did not affect the aortic annulus in either group, but did decrease the dimensions of the aortic root, sinotubular junction and ascending aorta. In general, statin therapy had a better effect on the aortas of the TAV patients than it did on those of the BAV patients.