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This study sought to assess the predictive value of wall shear stress (WSS) for colocalized ascending aorta (AAo) growth rate (GR) in patients with bicuspid aortic valve (BAV).BAV is associated with AAo dilation, but there is limited knowledge about possible predictors of aortic dilation in patients with BAV. An increased WSS has been related to aortic wall damage in patients with BAV, but no previous prospective study tested its predictive value for dilation rate. Recently, a registration-based technique for the semiautomatic mapping of aortic GR has been presented and validated.Forty-seven patients with BAV free from valvular dysfunction prospectively underwent 4-dimensional flow cardiac magnetic resonance to compute WSS and subsequent follow-up with 2 electrocardiogram-gated high-resolution contrast-enhanced computed tomography angiograms for GR assessment.During a median follow-up duration of 43 months, mid AAo GR was 0.24 mm/year. WSS and its circumferential component showed statistically significant association with mid AAo GR in bivariate (P = 0.049 and P = 0.014, respectively) and in multivariate analysis corrected for stroke volume and either baseline AAo diameter (P = 0.046 and P = 0.014, respectively) or z-score (P = 0.036 and P = 0.012, respectively). GR mapping further detailed that GR was heterogeneous in the AAo and that circumferential WSS, but not WSS magnitude, showed statistically significant positive associations with GR in the regions with the fastest growth.4D flow cardiac magnetic resonance-derived WSS and, in particular, its circumferential component predict progressive dilation of the ascending aorta in patients with BAV. Thus, the assessment of WSS may be considered in the follow-up of these patients.

Biallelic mutations in B3GALT6, coding for a galactosyltransferase involved in the synthesis of glycosaminoglycans (GAGs), have been associated with various clinical conditions, causing spondyloepimetaphyseal dysplasia with joint laxity type 1 (SEMDJL1 or SEMDJL Beighton type), Al-Gazali syndrome (ALGAZ), and a severe progeroid form of Ehlers-Danlos syndrome (EDSSPD2). In the 2017 Ehlers-Danlos syndrome (EDS) classification, Beta3GalT6-related disorders were grouped in the spondylodysplastic EDSs together with spondylodysplastic EDSs due to B4GALT7 and SLC39A13 mutations. Herein, we describe a patient with a previously unreported homozygous pathogenic B3GALT6 variant resulting in a complex phenotype more severe than spondyloepimetaphyseal dysplasia with joint laxity type 1, and having dural ectasia and aortic dilation as additionally associated features, further broadening the phenotypic spectrum of the Beta3GalT6-related syndromes. We also document the utility of repeating sequencing in patients with uninformative exomes, particularly when performed by using "first generations" enrichment capture methods.

Dilatació aòrtica; Aneurisma de l'aorta ascendent; Anàlisi espai-temporal Dilatación aórtica; Aneurisma de la aorta ascendente; Análisis espacio-temporal Aortic dilation; Ascending aorta aneurysm; Spatiotemporal analysis Motivated by the evidence that the onset and progression of the aneurysm of the ascending aorta (AAo) is intertwined with an adverse hemodynamic environment, the present study characterized in vivo the hemodynamic spatiotemporal complexity and organization in human aortas, with and without dilated AAo, exploring the relations with clinically relevant hemodynamic and geometric parameters. The Complex Networks (CNs) theory was applied for the first time to 4D flow magnetic resonance imaging (MRI) velocity data of ten patients, five of them presenting with AAo dilation. The time-histories along the cardiac cycle of velocity-based quantities were used to build correlation-based CNs. The CNs approach succeeded in capturing large-scale coherent flow features, delimiting flow separation and recirculation regions. CNs metrics highlighted that an increasing AAo dilation (expressed in terms of the ratio between the maximum AAo and aortic root diameter) disrupts the correlation in forward flow reducing the correlation persistence length, while preserving the spatiotemporal homogeneity of secondary flows. The application of CNs to in vivo 4D MRI data holds promise for a mechanistic understanding of the spatiotemporal complexity and organization of aortic flows, opening possibilities for the integration of in vivo quantitative hemodynamic information into risk stratification and classification criteria. Open access funding provided by Politecnico di Torino within the CRUI-CARE Agreement.

Background:Prior to the recent release of appropriate use criteria for imaging valvulopathies in children, follow-up of valvular lesions, including isolated bicuspid aortic valve, was not standardised. We describe current follow up, treatment, and intervention strategies for isolated bicuspid aortic valve with varying degrees of stenosis, regurgitation, and dilation in children up to 18 years old and compare them with newly released appropriate use criteria.Methods:Online survey was sent to members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and PediHeartNet.Results:Totally, 106 responses with interpretable data were received. For asymptomatic patients with isolated BAV without stenosis, regurgitation, or dilation follow-up-intervals increased from 7+/−4 months in the newborn period to 28 +/− 14 months at 18 years of age. Respondents recommended more frequent follow-up for younger patients and those with greater disease severity. More than 80% of respondents treat aortic regurgitation or aortic dilation in the setting of bicuspid aortic valve medically. In general, intervention was recommended once stenosis or regurgitation became severe (stenosis of >4 m/s; regurgitation with LV Z score 4) regardless of age, but was not routinely recommended for younger children (newborn – age 6 years) with severe dilation. Exercise was restricted at 38+/−11 mmHg echocardiographic mean gradient.Conclusions:Current follow-up, treatment, and intervention strategies for isolated bicuspid aortic valve deviate from appropriate use criteria. Differences between the two highlight the need to better delineate the disease course, clarify recommendations for care, and encourage wider adoption of guidelines.

Összefoglaló. Bevezetés: A Marfan-szindróma autoszomális domináns módon öröklődő, szisztémás kötőszöveti betegség. A hosszú távú túlélés szempontjából fontos a nagyérkatasztrófák megelőzése. Szívsebészeti szempontból a legfontosabb elváltozás az aortagyök tágulata. Aortagyök-rekonstrukciós beavatkozásaink Bentall–DeBono-, David I. és módosított Yacoub-műtétek, melyek mind preventív jelleggel, mind dissectio esetén jó eredménnyel végezhetők. Célkitűzés: A marfanos betegeknél eltérő technikával végzett aortagyök-rekonstrukciós műtéteink összehasonlítása. Módszer: A Semmelweis Egyetem Városmajori Szív- és Érgyógyászati Klinikáján 1993 és 2020 között Marfan-szindrómásoknál elvégzett Bentall–DeBono-, David I. és módosított Yacoub-féle aortagyök-rekonstrukciókat elemeztük. A műtét szerinti csoportok életkora a beavatkozás idején 29,69 (21,98–41,25) év, 29,15 ± 11,99 év és 35,29 ± 14,14 év volt, a fenti sorrendben. Az adatok forrásául a Magyar Marfan Regiszter és az Aortagyök-rekonstrukciós Regiszter szolgált. Eredmények: Az utánkövetési idő 132 ± 81,04 hónap volt a Bentall-, 76 ± 27,77 hónap a David-, valamint 4,5 (0,75–11,75) hónap a Yacoub-műtét esetén. A David- és a Yacoub-beavatkozások gyakrabban voltak profilaktikusak, mint a Bentall-operációk (p = 0,0153; p = 0,0085). A Bentall-műtéteknél ritkább volt a primer műtét esetleges későbbi elégtelenségéből fakadó reoperáció, mint a David-operációknál (pKövetkeztetés: Marfan-szindrómában a leggyakrabban halált okozó szövődmény az akut aortaruptura, illetve akut aortadissectio. Eredményeink alapján mindhárom profilaktikus aortagyök-rekonstrukciós műtéti típus jól reprodukálható és jó eredménnyel végezhető Marfan-szindrómában. Orv Hetil. 2021; 162(18): 696–704. Summary. Introduction: Marfan syndrome is an autosomal dominant, systemic connective tissue disorder. Preventing vascular complications is essential for long-term survival. Aortic dilation is the main cardiac surgical manifestation. Bentall–DeBono, David I and modified Yacoub aortic root reconstructions treat and prevent aortic dissections with great outcomes. Objective: Comparing results of aortic root reconstructions in Marfan syndrome. Method: We analysed the data of Bentall–DeBono, David I and modified Yacoub operations performed in Marfan syndrome at the Heart and Vascular Center, Semmelweis University between 1993 and 2020. Ages of surgical groups at the time of operation were 29.69 (21.98–41.25) years, 29.15 ± 11.99 years and 35.29 ± 14.14 years, respectively. Data were obtained from the Hungarian Marfan Register and the Aortic Root Reconstruction Register. Results: Follow-up time was 132 ± 81.04 months for Bentall, 76 ± 27.77 months for David and 4.5 (0.75–11.75) months for Yacoub groups. David and Yacoub operations were prophylactic more frequently than Bentall ones (p = 0.0153; p = 0.0085). Freedom from reoperation after primary surgery insufficiency was more common for Bentall than for David procedure (pConclusion: In Marfan syndrome, acute aortic rupture and dissection are the main contributors to mortality. Based on our results, the prophylactic aortic root reconstructions are reproducible and can be performed with great outcomes. Orv Hetil. 2021; 162(18): 696–704.

Background Aortic dilation has been associated with various cardiac conditions, although its prevalence and clinical correlates in hypertrophic cardiomyopathy (HCM) remain unclear. Objectives The purposes of this study were to define the prevalence of ascending aortic dilation in a large referral population of patients with HCM and to determine clinical and echocardiographic correlates of aortic dilation. Methods A total of 1,698 patients with HCM underwent echocardiographic measurement of the tubular ascending aorta (proximal and midlevel) during index evaluation at a tertiary HCM referral center. End-diastolic ascending aorta dimension was indexed to body surface area, with dilation defined for the tubular ascending aorta as 2 SD above the mean (>19 mm/m2) and independently as greater than published age-, sex-, and body surface area– adjusted norms (for the sinus of Valsalva and midlevel). Aortic size and presence of aortic enlargement were correlated with clinical and echocardiographic parameters. Results Tubular ascending aortic dilation >19 mm/m2 was present in 303 patients with HCM (18%), and dilation above adjusted norms was present in 210 patients with HCM (13%). The median indexed tubular ascending thoracic aortic dimension was 16.5 (interquartile range, 14.8-18.2) mm/m2. Indexed dimension increased linearly with age (R = 0.53, P 19 mm/m2 (29.8% vs 9.9% and 24.1% vs 10.5%, respectively, P 19 mm/m2 than those without resting or provocable obstruction (19.6% vs 14.4%, P = .007). Using adjusted norms, aortic enlargement was more frequent at the midlevel compared with the sinus of Valsalva (71% vs 29%), more common in patients with hypertension (15.4% vs 10.6%, P = .009), and more common in patients with paroxysmal atrial fibrillation (16.3% vs 11.5%, P = .036), but no other relationships remained statistically significant. Conclusions In this large cohort of patients with HCM, aortic dilation was common. The key correlate of tubular aortic enlargement >19 mm/m2, and aortic enlargement greater than adjusted norms included a history of systemic hypertension. Given an increased prevalence of aortic dilation in HCM, further study is needed on the clinical impact of aortic dilation.

Background:Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients.Methods and results:From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]).Conclusions:Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

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 cm2 /year, with 30% of bicuspid aortic valve patients progressing ≥0.1 cm2 /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.

While the immediate effects of pregnancy on aortic dimension in patients with Marfan syndrome (MFS) have been evaluated, the late effects of subsequent pregnancies in these patients are less known. For this purpose, we evaluated 2 groups of women with MFS who were under specialized care in our institution. Group A included 23 women with MFS who experienced 55 pregnancies; group B included 12 nulliparous MFS patients. Patients in group A were similar in age (36.13 ± 5.6 years vs. 34.25 ± 6.54 years, p = 0.41) and follow-up time (group A 6.05 ± 3.56 years and group B 4.92 ± 3.37 years, p = 0.37). Baseline aortic root diameters as well as the aortic root diameters at follow-up visits were similar between groups (35.60 ± 4.42 vs. 35.08 ± 3.82 mm, p = 0.73, and 37.57 ± 4.66 vs. 37.33 ± 4.83 mm, p = 0.89, respectively). The aortic root diameter increased by 0.5 (0, 2) mm in group A and 1 (0, 4.5) mm in group B (p = 0.54). The rate of aortic dilation per year of follow-up was similar between the groups (0.34 ± 0.52 mm/year in group A vs. 0.55 ± 0.75 mm/year in group B [p = 0.52]). Chronic medical treatment was similar in both groups. Patients in both groups were treated similarly with β-blockers, angiotensin-converting enzyme inhibitors, and angiotensin II receptor blockers. In summary, subsequent pregnancies in patients with MFS were not associated with an increase in the rate of aortic root dilation in these patients.

Loeys-Dietz syndrome (LDS) type 3 results from a SMAD3 mutation and is a phenotypically milder variant of LDS with frequent aortic, visceral, and cerebral vascular pathologies and osteoarthritis. Historically, endovascular treatment (endovascular aortic repair [EVAR]) of LDS-related aortic aneurysmal disease with traditional modular bifurcated devices has been limited owing to concerns regarding continued aortic dilation at proximal fixation sites. Furthermore, associated dissection pathology has also precluded traditional modular bifurcated EVAR owing to inadequate proximal infrarenal necks and narrow distal aortic domains leading to compromised contralateral gate opening and cannulation as well as limb flow compromise. To address these barriers to EVAR, we present our approach for the endovascular treatment of a dissecting pararenal abdominal aortic aneurysm using an anatomically fixated, bifurcated, unibody aortic stent graft in a patient with LDS-3.

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. 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. 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. 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.

A computational hemodynamics method was employed to investigate how the morphotype and functional state of aortic valve would affect the characteristics of blood flow in aortas with pathological dilation, especially the intensity and distribution of flow turbulence. Two patient-specific aortas diagnosed to have pathological dilation of the ascending segment while differential aortic valve conditions (i.e., one with a stenotic and regurgitant RL bicuspid aortic valve (RL-BAV), whereas the other with a quasi-normal tricuspid aortic valve (TAV)) were studied. When building the computational models, in addition to in vivo data-based reconstruction of geometrical model and boundary condition setting, the large eddy simulation method was adopted to quantify potential flow turbulence in the aortas. Obtained results revealed the presence of complex flow patterns (denoted by time-varying changes in vortex structure), flow turbulence (indicated by high turbulent eddy viscosity (TEV)), and regional high wall shear stress (WSS) in the ascending segment of both aortas. Such hemodynamic characteristics were significantly augmented in the aorta with RL-BAV. For instance, the space-averaged TEV in late systole and the wall area exposed to high time-averaged WSS (judged by WSS> two times of the mean WSS in the entire aorta) in the ascending aortic segment were increased by 176% and 465%, respectively. Relatively, flow patterns in the descending aortic segment were less influenced by the aortic valve disease. These results indicate that aortic valve disease has profound impacts on flow characteristics in the ascending aorta, especially the distribution and degree of high WSS and flow turbulence.

A bicuspid aortic valve (BAV) is a congenital heart defect in which the heart valve between the left ventricle and the aorta consists of two valve flaps instead of the normal three (tricuspid aortic valve – TAV). In a large proportion of people with a BAV, calcification of the heart valves or a widening of the aorta occurs early in life. It is not yet clear why patients with a BAV are prone to develop valvular calcification and aortic dilatation. The aim of the research described in this thesis is to study the pathogenesis of aortic valve calcification and aortic dilatation in BAV patients with a focus on the role of endothelial cells in these processes. With the research described in this thesis, we have shown that endothelial cells from BAV patients respond differently than cells from people with a TAV. In addition, the endothelial cell activation in the vascular wall of BAV patients is different and dependent on the blood flow. We found two good tissue culture methods to study heart valve calcification and used them to study the role of the protein FHL2 in this process.

Purpose: This study aimed to investigate the effect of distal aortic segmental enlargement (DASE) after thoracic endovascular aortic repair for complicated type B aortic dissection (cTBAD). Materials and Methods: From March 2003 to October 2018, 814 patients with acute cTBAD from 5 medical centers were retrospectively identified. DASE is indicated as the enlargement of distal aortic segmental volume ≥1.6 fold of the preoperative volume compared with the most recent postoperative computed tomography angiography (CTA) scan. Of these patients, 635 (78%) were identified as non-DASE, and 179 (22%) were identified as DASE. Competing risk analysis was performed to compare late death and distal aortic reintervention between the groups. The morphological variables and false lumen thrombosis at 7 aortic levels were measured based on the preoperative CTA and the most recent CTA. Univariate and multivariate Cox regression analyses were used to assess the independent predictors of DASE. Results: The mean follow-up time of the entire cohort was 5.6 years (interquartile range: 2.4–8.3 years). There were total of 208 late deaths, including 94 (14.8%) deaths in non-DASE group versus 114 (63.7%) deaths in the DASE group. Distal aortic reintervention was observed in 89 patients, with 43(6.7%) in the non-DASE group versus 46 (25.7%) in the DASE group. The cumulative incidence of late death and distal aortic reintervention were significantly higher in the DASE than in the non-DASE group (pConclusion: Compared with the non-DASE group, patients with DASE demonstrated a higher rate of late death and distal aortic reintervention. For the cTBAD population, extended stent-graft coverage to the aortic section between diaphragm and celiac artery might serve as a “cost-efficient” cutoff point aiming to reduce the risk of DASE.

OBJECTIVES This study investigated the impact of aortic diameter on late aortic dilation of the residual dissected aorta after tear-oriented aortic replacement for acute DeBakey type I aortic dissection. METHODS Of 133 patients who underwent aortic replacement for acute DeBakey type I/II aortic dissection between 2008 and 2019, 45 patients with a residual dissected aorta after surgery for acute DeBakey type I aortic dissection and who underwent computed tomography at predischarge and after 1 year were retrospectively assessed. The aortic diameter and false lumen area were measured at three levels: the maximal aortic site, seventh thoracic vertebra, and celiac axis. Multivariable Cox regression analysis was employed to identify the predictors of late aortic dilation, defined as an aortic growth rate of ≥ 5 mm/year or a maximal aortic diameter of ≥ 55 mm. RESULTS During a median follow-up of 75 [range: 13-152] months, 6 patients (5 men; mean age: 57 ± 14 years) experienced aortic dilation. All 6 patients had the maximal aortic diameter between the distal aortic arch and seventh thoracic vertebra level at the last computed tomography. Multivariable Cox regression analysis showed that the predischarge maximal aortic diameter was an independent determinant of late aortic dilation (hazard ratio: 2.28/mm, 95% confidence interval: 1.10-5.86). CONCLUSIONS Predischarge maximal aortic diameter is a significant predictor of late aortic dilation in patients with a residual dissected aorta after tear-oriented surgical repair of acute DeBakey type I aortic dissection.

Background Data from the International Registry of Acute Aortic Dissection indicate that the guideline criterion of 5.5 cm for ascending aortic intervention misses many dissections occurring at smaller dimensions. Furthermore, studies of natural behavior have generally treated the aortic root and the ascending aorta as 1 unit despite embryological, anatomical, and functional differences. This study aims to disentangle the natural histories of the aforementioned aortic segments, allowing natural behavior to define specific intervention criteria for root and ascending segments of the aorta. Methods and Results Diameters of the aortic root and mid‐ascending segment were measured separately. Long‐term complications (dissection, rupture, and death) were analyzed retrospectively for 1162 patients with ascending thoracic aortic aneurysm. Cox regression analysis suggested that aortic root dilatation ( P =0.017) is more significant in predicting adverse events than mid‐ascending aortic dilatation ( P =0.087). Short stature posed as a serious risk factor. The dedicated risk curves for the aortic root and the mid‐ascending aorta revealed hinge points at 5.0 and 5.25 cm, respectively. Conclusions The natural histories of the aortic root and mid‐ascending aorta are uniquely different. Dilation of the aortic root imparts a significant higher risk of adverse events. A diameter shift for intervention to 5.0 cm for the aortic root and to 5.25 cm for the mid‐ascending aorta should be considered at expert centers.

Tutors: Andrea Guala, Oscar Camara Aortic aneurysms are a class of frequent cardiovascular disease related to an increased risk of life-threating complications, such as aortic dissection and rupture. These acute events are characterized by a very bad prognosis, with mortality comprised between 60 to 70%. Maximum aortic diameter is the sole established parameter for risk assessment but many acute events happen at a diameter lower than that suggested for preventive substitution of the diseased region. Aortic deformation may be used for better risk stratification. Three-dimensional (3D) time-resolved cine cardiac magnetic resonance images (CMR) allows for the quantification of strain over the totality of the aorta, overcoming many limitations of 2D imaging, such as the predefined location of analysis and through plane deformation. The aim of the present study was to assess 4D strain of the whole thoraco-abdominal aorta during one cardiac cycle. The dataset was provided by Hospital Universitari Vall d’Hebron and consisted on 3D cine CMR and contrast-enhanced 3D angiography (MRA). 3D volume of the whole thoraco-abdominal aorta was obtained from contrast-enhanced 3D angiography and allows to identify the aortic wall and extract deformation values at those points. Volumetric images of the aorta from 3D cine CMR at different cardiac phases were registered and the deformation field at the aortic wall was extracted and analysed. Local strain of the wall was extracted in anatomically-identified regions and was divided into circumferential and radialcomponents. The evaluation of aortic strain may help to monitor and evaluate risk in patients with aortic aneurysms.

Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. BAV aortic dilatation is associated with an increased risk of adverse aortic events and represents a potentially lethal disease and hence a considerable medical burden. BAV with aortic dilatation warrants frequent monitoring, and elective surgical intervention is the only effective method to prevent dissection or rupture. The predictive value of the aortic diameter is known to be limited. The aortic diameter is presently still the main reference standard for surgical intervention owing to the lack of a comprehensive understanding of BAV aortopathy progression. This article provides a brief comprehensive review of the current knowledge on BAV aortopathy regarding clinical definitions, epidemiology, natural course, and pathophysiology, as well as hemodynamic and clinically significant aspects on the basis of the limited data available.

Aortic dilation has been increasingly recognized in congenital heart diseases, and aortic dissection is one of the important complications. We report a case of aortic dissection in a patient 31 years after repair of tetralogy of Fallot (TOF) and review reported cases. While aortic dissection is uncommon, aortic dilation is common among patients with repaired TOF and it appeared progressive in some patients. Based on the reported cases, progressive aortic dilation appeared as the pre-requisite for aortic dissection, although other factors might be involved. Regular surveillance and monitoring for aortic complications should be incorporated into clinical practice.

Supplemental Digital Content is available in the text., Objective: To assess the relationship between regional wall shear stress (WSS) and oscillatory shear index (OSI) and aortic dilation in patients with bicuspid aortic valve (BAV). Approach and Results: Forty-six consecutive patients with BAV (63% with right-left-coronary-cusp fusion, aortic diameter ≤ 45 mm and no severe valvular disease) and 44 healthy volunteers were studied by time-resolved 3-dimensional phase-contrast magnetic resonance imaging. WSS and OSI were quantified at different levels of the ascending aorta and the aortic arch, and regional WSS and OSI maps were obtained. Seventy percent of BAV had ascending aorta dilation. Compared with healthy volunteers, patients with BAV had increased WSS and decreased OSI in most of the ascending aorta and the aortic arch. In both BAV and healthy volunteers, regions of high WSS matched regions of low OSI and vice versa. No regions of both low WSS and high OSI were identified in BAV compared with healthy volunteers. Patients with BAV with dilated compared with nondilated aorta presented low and oscillatory WSS in the aortic arch, but not in the ascending aorta where dilation is more prevalent. Furthermore, no regions of concomitant low WSS and high OSI were identified when BAV were compared according to leaflet fusion pattern, despite the well-known differences in regional dilation prevalence. Conclusions: Regions with low WSS and high OSI do not match those with the highest prevalence of dilation in patients with BAV, thus providing no evidence to support the low and oscillatory shear stress theory in the pathogenesis of proximal aorta dilation in the presence of BAV.

Key Clinical Message We present a patient with Phelan‐McDermid syndrome, a rare neurodevelopmental disorder caused by a 22q13 deletion, with the previously undescribed finding of progressive dilation of the great arteries. While congenital heart defects have been identified in patients previously, dilation of the great arteries has not been described to our knowledge.

We report three patients with Feingold 2 syndrome with the novel features of growth hormone deficiency associated with adenohypophyseal compression, aortic dilation, phalangeal joint contractures, memory, and sleep problems in addition to the typical features of microcephaly, brachymesophalangy, toe syndactyly, short stature, and cardiac anomalies. Microdeletions of chromosome 13q that include the MIR17HG gene were found in all three. One of the patients was treated successfully with growth hormone. In addition to expanding the phenotype of Feingold 2 syndrome, we suggest management of patients with Feingold 2 syndrome include echocardiography at the time of diagnosis in all patients and consideration of evaluation for growth hormone deficiency in patients with short stature.

Background: There is a growing interest in the relationship between atrial septal anatomy and cardioembolic stroke. Anecdotal reports suggest that the enlargement of the aortic root could interfere with atrial septal mobility (ASM). We sought to investigate the association between ASM and aortic root dilation.Methods and Findings: From all consecutive clinically requested transesophageal echocardiogram (TEE) studies performed during the study period in a single institution, we were able to review and evaluate the ASM and anteroposterior length, aortic root diameter, and the prevalence of atrial septal aneurysm (ASA) and of patent foramen ovale (PFO) in 336 studies. Additional variables, such as left ventricular ejection fraction, left atrial diameter, diastolic dysfunction, age, sex, weight, height, previous stroke, atrial fibrillation, and TEE indication, were extracted from patient medical records and echocardiographic clinical reports. In 336 patients, we found a mean ASM of 3.4 mm, ranging from 0 to 21 mm; 15% had ASA and 14% had PFO. There was a 1.0 mm increase in ASM for every 10-mm increase in aortic root diameter adjusted for age, sex, weight, height, ejection fraction, and left atrial size (B = 0.1; P = 0.04). Aortic diameter was not associated with a smaller septal length (B = 0.03; P = 0.7).Conclusion: An increased motion of the atrial septum can occur in association with aortic dilation. These findings deserve attention for the relevance of aortic root anatomy in future studies involving atrial septal characteristics and embolic stroke risk.

ObjectiveAortic ruptures are fatal consequences of aortic aneurysms with macrophage accumulation being a hallmark at the site of ruptures. Pyroptosis is critical in macrophage-mediated inflammation. This study determined effects of pyroptosis on aortic dilation and rupture using GSDMD deficient mice.Approach and ResultsIn an initial study, male Gsdmd+/+ and Gsdmd-/- mice in C57BL/6J background (8 – 10 weeks old) were infected with adeno-associated viral vectors encoding mouse PCSK9D377Y gain-of-function mutation and fed a Western diet to induce hypercholesterolemia. After two weeks of AAV infection, angiotensin II (AngII, 1 µg/kg/min) was infused. During the 4 weeks of AngII infusion, 5 of 13 Gsdmd+/+ mice died of aortic rupture, whereas no aortic rupture occurred in Gsdmd-/- mice. In surviving mice, no differences in either ascending or abdominal aortic dilation were observed between Gsdmd+/+ and Gsdmd-/- mice. To determine whether protection of GSDMD deficiency against aortic rupture is specific to AngII infusion, we subsequently examined aortic pathologies in mice administered beta-aminopropionitrile (BAPN). BAPN (0.5% wt/vol) was administered in drinking water to male Gsdmd+/+ and Gsdmd-/- mice (4 weeks old) for 4 weeks. Six of 13 Gsdmd+/+ mice died of aortic rupture, whereas no aortic rupture occurred in Gsdmd-/- mice. In mice survived, no differences of diameters in the ascending, arch, or abdominal aortic regions were observed between Gsdmd+/+ and Gsdmd-/- mice.ConclusionsGSDMD deficiency protects against AngII or BAPN-induced aortic ruptures in mice.HighlightsGSDMD deficiency protects against angiotensin II-induced aortic rupture in hypercholesterolemic mice.GSDMD deficiency protects against beta-aminopropionitrile (BAPN)-induced aortic dissection and rupture in C57BL/6J mice.

Normal aortic valve has three leaflets. Quadricuspid aortic valve(QAV) is a rare congenital anomaly of aortic valve in which there are four cusps. These patients should be diagnosed and clinically monitored as they are prone to developing aortic regurgitation in their life. Other structural anomalies and aortic dilation are also common in these patients. Advances in cardiac imaging have resulted in more and earlier detection of QAV.

Funding Acknowledgements Type of funding sources: None. Background The natural history of thoracic aortic aneurysm (TAA) is one of progressive expansion. Asymptomatic patients who do not meet criteria for repair require conservative management including ongoing aneurysm surveillance, mostly carried out by contrast-enhanced computed tomography angiography (CTA). Purpose To prospectively compare image quality and reliability of a prototype non-contrast, self-navigated 3D whole-heart magnetic resonance angiography (MRA) with contrast-enhanced computed tomography angiography (CTA) for sizing of thoracic aortic aneurysm (TAA). Methods Self-navigated 3D whole-heart 1.5 T MRA was performed in 20 patients (aged 67 ± 8.6 years, 75% male) for sizing of TAA; a subgroup of 18 (90%) patients underwent additional contrast-enhanced CTA on the same day. Subjective image quality was scored according to a 4-point Likert scale and ratings between observers were compared by Cohen’s Kappa statistics. Continuous MRA and CTA measurements were analyzed with regression and Bland-Altman analysis. Results Overall subjective image quality as rated by two observers was 1 [interquartile range (IQR) 1-2] for self-navigated MRA and 1.5 [IQR 1-2] for CTA (p = 0.717). For MRA a perfect inter-observer agreement was found for presence of artefacts and subjective image sharpness (κ=1). Subjective signal inhomogeneity correlated highly with objectively quantified inhomogeneity of the blood pool signal (r = 0.78-0.824, all p Conclusion Self-navigated 3D whole-heart MRA enables reliable contrast- and radiation free aortic dilation surveillance without significant difference to standardized CTA while providing predictable acquisition time and by offering excellent image quality. Abstract Figure.

Introduction: Guideline recommendations of prophylactic surgery in ascending aortic dilation by maximum aortic diameter (MAD) fail to predict > 50% of type-A dissections (AD). Assessment of post-dissection diameters as reference and lack of somatometric normalization may preclude an appropriate risk estimation. Hypothesis: The combined assessment of Z-score and Svensson-index (cross-area/height) based on pre-dissection aortic diameters may be advantageous to indicate prophylactic ascending aortic replacement. Such an approach would include information on normalized vascular dilatation together with a clinical aortic risk indicator. Methods: During two years (2018-2019), data from 515 AD-patients were prospectively recorded at 32 tertiary Spanish hospitals (Registro Español Síndrome Aórtico-III). Pre-dissection aortic diameters were estimated based on the in vitro observations made by Williams et al. on the perimeter change of normal human aortas after the creation of a dissection (PMID: 9122399). Svensson indexes were correlated with ascending aorta Z-scores using quadratic regression. Results (Figure): Setting thresholds of increased risk at Svensson-index >10 cm 2 /m and aortic dilation at Z-score >3, 59% of patients had low Svensson and low Z-score category, 19% low Svensson but high Z-score and 22% high Svensson and high Z-score. No patient with Svensson-index 2 /m and Z-score either < or > 3 had an indication for surgery according to guidelines. Among patients with Svensson-index >10 cm 2 /m and aortic dilation at Z-score >3, approximately 1/3 (32%) would have a surgical indication whereas 2/3 (68%) would not. Conclusions: According to current guidelines, only one third of high Svensson and high Z group (7% of the total cohort) would deserve elective surgery. More proactive guidelines, suggesting replacement of ascending aorta in patients with Svensson-index >10 and Z-score>3, would spare from dissection 22% of current cases.

Introduction: Variation in aortic root (AoR) rotational position affects flow dynamics in normal patients. Complications of arterial switch operation (ASO) for transposition of the great arteries (TGA) include aortic dilation and neo-aortic regurgitation (AR). We sought to assess the association of neo-AoR rotational position with neo-AoR and ascending aorta (AA) dilation and neo-AR in TGA after ASO. Methods: Patients after ASO for TGA who underwent cardiac magnetic resonance (CMR) from 2005-2020 were retrospectively reviewed. Neo-AoR rotational angle, indexed (to height) neo-AoR and AA dimensions, indexed left ventricular end diastolic volume (LVEDVi), and neo-aortic regurgitant fraction (RF) were obtained from CMR. Multivariable regression analysis for neo-AoR and AA dilation, RF, and LVEDVi were performed. Results: A total of 36 patients (78% male) were included. Median age at ASO was 5 days (3, 7) and at CMR was 17.1 years (12.3, 21.9). Rotational angle was 25% counterclockwise (+15°). There was no material association of neo-AoR and AA size with sex, age, and coronary artery anatomy. A quadratic term for neo-AoR rotational angle - increasing extremes of counterclockwise and clockwise angles - was significantly associated with neo-AoR dilation (p=0.03), AA dilation (p Conclusions: In conclusion, both clockwise and counterclockwise neo-AoR rotational position in TGA after ASO are associated with neo-AoR and AA dilation and LVEDVi. Counterclockwise rotational angle was associated with RF. Neo-AoR rotational position likely affects valve function and hemodynamics, leading to risk of neo-AR and aortic dilation.

Myocardial adaptation to athletics and exercise training is a well-described process that has garnered much interest over the past decades. By contrast, our understanding of how the vascular system adapts in the trained athlete remains limited. The goal of this review is to provide a comprehensive overview of the impact of exercise on structure and function of the extra-cardiac vascular system. While structural changes in the size of the aorta are rare in young athletes, emerging data suggest that long-term athletic participation may be a risk factor for aortic dilation in middle-age and beyond. In addition, new findings show that even modest amounts of athletic training such as participation in an individual’s first marathon can have important salutary effects on vascular health. Vascular adaptations represent an important part of the athletic phenotype and likely play an important role in the overall cardiovascular health of the trained athlete.

Patients with subaneurysmal aortic dilation (SAD, 25&ndash, 29 mm diameter) are likely to progress to true abdominal aortic aneurysm (AAA). Despite these patients having a higher risk of all-cause mortality than subjects with aortic size &lt, 24 mm, early diagnostic biomarkers are lacking. MicroRNAs (miRs) are well-recognized potential biomarkers due to their differential expression in different tissues and their stability in blood. We have investigated whether a plasma miRs profile could identify the presence of SAD in high cardiovascular risk patients. Using qRT-PCR arrays in plasma samples, we determined miRs differentially expressed between SAD patients and patients with normal aortic diameter. We then selected 12 miRs to be investigated as biomarkers by construction of ROC curves. A total of 82 significantly differentially expressed miRs were found by qPCR array, and 12 were validated by qRT-PCR. ROC curve analyses showed that seven selected miRs (miR-28-3p, miR-29a-3p, miR-93-3p, miR-150-5p, miR-338-3p, miR-339-3p, and miR-378a-3p) could be valuable biomarkers for distinguishing SAD patients. MiR-339-3p showed the best sensitivity and specificity, even after combination with other miRs. Decreased miR-339-3p expression was associated with increased aortic abdominal diameter. MiR-339-3p, alone or in combination with other miRs, could be used for SAD screening in high cardiovascular risk patients, helping to the early diagnosis of asymptomatic AAA.

The risks of ascending aortic dilation and indications for intervention in pediatric patients are unclear. Given the concern for aortic size mismatch with growth, larger ascending aortic diameters have been accepted in pediatric patients. The purpose of this study was to evaluate the effectiveness of ascending aortic reduction at the time of aortic valve surgery and its effect on recurrent aortic regurgitation.A retrospective 8-year observational review (2010-2018) was conducted in pediatric patients with dilated ascending aorta undergoing aortic valve surgery at a single institution.Forty-seven patients underwent ascending aortoplasty during aortic valve surgery, of which 39 patients with complete data points had preoperative mean ascending aortic Z-scores of 5.35 ± 1.52 reduced to 1.22 ± 1.63 (P.001) postoperatively. Ascending aortic Z-scores remained similar at 1.37 ± 1.72 at latest follow-up (P = .306). In contrast our matched control group (n = 39) without ascending aortoplasty had preoperative mean ascending aortic Z-scores of 4.15 ± 1.65 reduced to 3.26 ± 2.0 at the midterm follow-up. This reduction was significantly larger in the aortoplasty group, with an average difference in these changes of 3.12 (95% confidence interval, 2.43-3.8; P.001). Multivariable logistic regression (controlling for aortic valve intervention type and body surface area) showed a 6.84 odds of moderate to severe aortic regurgitation at follow-up in the control group compared with the aortoplasty group (95% confidence interval, 1.43-32.8; P = .016). There were no complications associated with ascending aortoplasty.Ascending aortoplasty is effective in reducing ascending aortic dimensions and recurrent aortic regurgitation in pediatric patients. Longer-term follow-up is necessary to determine continued rate of growth.

BACKGROUND: Female sex protects against abdominal aortic aneurysms (AAAs); however, the mechanisms behind these sex-based differences remain unknown. The purpose of this study was to explore the role of sex and sex hormones in AAA formation among swine. MATERIALS AND METHODS: Using a previous validated model, infrarenal AAA were surgically created in uncastrated male (n=8), female (n=5), and castrated male (n=4) swine. Aortic dilation was measured on post-operative day 28 during the terminal procedure and compared to initial aortic diameter measured during the index procedure. Tissue was analyzed for immunohistochemistry, cytokine array, gelatin zymography, serum 17β–Estradiol, and testosterone assay. RESULTS: Uncastrated males had significantly larger maximal aortic dilation compared to castrated males (113.5%±11.4% vs 38.1%±4.5%, p=.0012). Females had significantly higher mean aortic dilation compared to castrated males (96.2%±7.5% vs 38.1%±4.5%, p=.0004). Aortic diameters between females and uncastrated males were not significantly different on day 28. Female swine had significantly higher concentrations of 17β-estradiol compared with uncastrated males (1590±873.3 ng/mL vs 95.2±2.3 ng/mL, p=.047), with no significant difference between females and castrated males. Uncastrated male AAA demonstrated significantly more elastin degradation compared with female and castrated males (p=0.01 and

Cardiovascular diseases remain the leading cause of death in OECD countries, in particular, because of the population aging, making it one of the major health issues on a global scale. Advances in imaging today make it possible to better understand and diagnose these diseases in a non-invasive way. More recently, a new non-invasive and non-radiative imaging technique named « 4D Flow MRI » allows for the first time to image the speed of blood flow in three dimensions during a whole cardiac cycle, thus offering new perspectives of visualization, understanding, and measurement. This thesis in image processing carried out in connection with cardiologists and radiologists aims to develop new indicators and to quantify the contribution of 4D flow MRI especially in 1) the assessment of the aortic stiffness leading to a comparison between several approaches to estimate the pulse wave velocity 2) the analysis of flow disorganization in aging and pathological dilation 3) the evaluation of filling flow in the left ventricle.; Les maladies cardiovasculaires sont la première cause de mortalité dans les pays de l’OCDE du fait notamment du vieillissement global de la population, faisant d’elles l’un des enjeux majeurs de santé à l’échelle mondiale. Les progrès de l’imagerie permettent aujourd’hui de mieux comprendre et diagnostiquer ces maladies de manière non-invasive. Plus récemment, une technique novatrice d’imagerie non-invasive et non-irradiante appelée « IRM de flux 4D » permet pour la première fois d’imager la vitesse du flux sanguin en 3 dimensions pendant tout un cycle cardiaque, offrant ainsi de nouvelles perspectives de visualisation, de compréhension et de mesure. Cette thèse en traitement d’images, réalisée en lien avec des cardiologues et radiologues a pour objectif de développer de nouveaux indicateurs, pour quantifier l’apport de l’IRM de flux 4D notamment dans :1) l’évaluation de la rigidité aortique où une comparaison de méthodes pour l’évaluation de la vitesse de l’onde de pouls a été réalisée 2) l’analyse de la désorganisation de flux dans la dilatation aortique physiologique (âge) et pathologique ; 3) l’évaluation des flux de remplissage cardiaques.

Background Aortic dilatation is a cardiovascular complication in pediatric renal transplant recipients and may have an increased risk of aortic dissection, aortic rupture, and death. Studies failed to show an association between blood pressure and aortic dilatation; however, 24-hours ambulatory blood pressure monitoring (ABPM) was not performed. There was also no comparison between preemptive transplantation and dialysis. Methods After ethics approval, a retrospective cross-sectional study was performed on all prevalent pediatric renal transplant recipients from a single tertiary care center. The presence of aortic dilatation was determined using standard echocardiographic measurements, and those with other risk factors for aortic dilatation were excluded. Associations between 24-hours ABPM, renal function, dialysis history, and aortic dimensions were determined. Results We enrolled 37 participants with the following characteristics: 46% female, mean age 14.5 ± 3.7 years, 16% preemptive transplantation, and median end-stage renal disease (ESRD) combined vintage (time from ESRD onset to echocardiogram) 597 days (range 289-1290 days). We found 16/37 patients (43%) with aortic dilatation at any level, mostly mild. There was no association between 24-hours ABPM measurements and aortic dilatation. None of the preemptively transplanted children had aortic dilatation. Conclusion This study confirms a high prevalence of aortic dilatation among pediatric renal transplant recipients, which appears to be independent of hypertension on 24-hour ABPM. Patients with preemptive renal transplantation did not have aortic dilatation, suggesting that the effects of dialysis may contribute to the high prevalence of this complication. Pediatric cardiologists need to carefully assess aortic dimensions in these at-risk patients.

Objective: The combination of aortic coarctation (CoA) and bicuspid aortic valve (BAV) is assumed to be associated with a higher risk of ascending aortic dilatation and type A dissection, and current European Society of Cardiology (ESC) guidelines advise therefore to operate at a lower threshold in the presence of CoA. The aim of our study is to evaluate whether the coexistence of CoA in BAV patients is indeed associated with a higher risk of ascending aortic events (AAE). Methods: In a retrospective study, all adult BAV patients visiting the outpatient clinic of our tertiary care center between February 2003 and February 2019 were included. The primary end point was an ascending aortic event (AAE) defined as ascending aortic dissection/rupture or preventive surgery. The secondary end points were aortic dilatation and aortic growth. Results: In total, 499 BAV patients (43.7% female, age 40.3 &plusmn, 15.7 years) were included, of which 121 (24%) had a history of CoA (cBAV). An aortic event occurred in 38 (7.6%) patients at a mean age of 49.0 &plusmn, 13.6 years. In the isolated BAV group (iBAV), significantly more AAE occurred, but this was mainly driven by aortic valve dysfunction as indication for aortic surgery. There was no significant difference in the occurrence of dissection or severely dilated ascending aorta (&gt, 50mm) between the iBAV and cBAV patients (p = 0.56). The aortic diameter was significantly smaller in the cBAV group (30.3 &plusmn, 6.9 mm versus 35.7 &plusmn, 7.6 mm, p &lt, 0.001). The median aortic diameter increase was 0.23 (interquartile range (IQR): 0.0&ndash, 0.67) mm/year and was not significantly different between both groups (p = 0.74). Conclusion: Coexistence of CoA in BAV patients was not associated with a higher risk of aortic dissection, preventive aortic surgery, aortic dilatation, or more rapid aorta growth. This study suggests that CoA is not a risk factor in BAV patients, and the advice to operate at lower diameter should be reevaluated.

Background. Some adult patients with Tetralogy of Fallot (TOF) were found to simultaneously develop ascending aortic dilation. Severe aortic dilation would lead to several aortic diseases, including aortic aneurysm and dissection, which seriously affect patients’ living quality and even cause patients’ death. Current practice guidelines of aortic-dilation-related diseases mainly focus on aortic diameter, which has been found not always a good indicator. Therefore, it may be clinically useful to identify some other factors that can potentially better predict aortic response to dilation. Methods. 20 TOF patients scheduled for TOF repair surgery were recruited in this study and were divided into dilated and nondilated groups according to the Z scores of ascending aorta diameters. Patient-specific aortic CT images, pressure, and flow rates were used in the construction of computational biomechanical models. Results. Simulation results demonstrated a good coincidence between numerical mean flow rate at inlet and the one obtained from color Doppler ultrasonography, which implied that computational models were able to simulate the movement of the aorta and blood inside accurately. Our results indicated that aortic stress can effectively differentiate patients of the dilated group from the ones of the nondilated group. Mean ascending aortic stress-P1 (maximal principal stress) from the dilated group was 54% higher than that from the nondilated group (97.97 kPa vs. 63.47 kPa, p value = 0.044) under systolic pressure. Velocity magnitude in the aorta and aortic wall displacement of the dilated group were also greater than those of the nondilated group with p value < 0.1. Conclusion. Computational modeling and ascending aortic biomechanical factors may be used as a potential tool to identify and analyze aortic response to dilation. Large-scale clinical studies are needed to validate these preliminary findings.

Background: Patients with a bicuspid aortic valve (BAV) have an increased risk for aortic dilation and dissection. In this study, we provide a histological stratification of the developing aorta in the tricuspid aortic valve (TAV) and the BAV populations as a reference for future studies on aortopathy and related syndromes. Methods: Non-dilated TAV and BAV ascending aortic wall samples were collected, including 60 TAV (embryonic&ndash, 70 years) and 32 BAV specimens (fetal&ndash, 72 years, categorized in eight age groups. Results: In TAV, intimal development starts in the neonatal phase. After birth, the thickness of the medial layer increases significantly by increase of elastic lamellae up to and including the &ldquo, young child&rdquo, phase stabilizing afterwards. The BAV shows already prenatal intimal thickening becoming significantly thinner after birth subsequently stabilizing. In BAV, increase in elastic lamellae is seen between the young child and the adolescent phases, stabilizing afterwards. Conclusions: Vascular development in TAV is described in three phases: maturation, stabilization, and degeneration. For BAV, the development can be described in two phases: maturation (already prenatally) and degeneration. After birth, the development of the aorta is characterized by degeneration, leading to weakening of the ascending aortic wall and increasing the risk of aortopathy.

Introduction: Morphology of bicuspid aortic valve (BAV) may have implication in the associated pathologies including aortic stenosis (AS), aortic insufficiency (AI) and aortic dilation. The aim of this study is to investigate the frequency and patterns of valvular dysfunction and aortopathy associated with different phenotypes of BAV in a referral center in northwest of Iran. Methods: In this prospective study patients who presented to our echocardiography lab between January 2014 and December 2015 and were diagnosed with BAV were assessed. Frequency of various BAV phenotypes and their association with valvular dysfunction and aortopathy was evaluated. A P value less than 0.05 was considered statistically significant. Results: The average age of the study patients was 40±16 years, with predominance of male sex (72%). Patients with anteroposteriorly located BAV (BAV-AP) phenotype constituted majority of our cases with prevalence of 62.7%, while 37.3% of cases had right-left (BAV-RL) located valves. BAV-RL patients when compared to BAV-AP patients had higher frequencies of dilated aortic arch (25% vs. 4.3%, P

Background Type B intramural hematoma (IMH) is considered a low-risk entity for aortic complications if aortic dilation, containing rupture or clinical instability are absent. However, the development of intimal disruptions (ID), present in >40% of cases, poses an unknown risk. Objectives To establish which ID characteristics imply a higher risk of aortic complications and, therefore, merit invasive treatment. Methods A systematic review and a meta-analysis were made following a search in EMBASE, MEDLINE and PsycINFO for articles published between January 1995 and December 2017. The combined endpoint was defined as aortic mortality, invasive treatment for aortic disease and/or increase in maximum aortic diameter ≥55 mm. Lesions with communicating orifice ≤3 mm were defined as tiny ID (TID) and those with >3 mm as focal ID (FID). Results Six studies with 564 participants diagnosed of type B IMH were included. Incidence of ID was 54.3% (306 individuals): 27.7% (156 individuals) initially met TID criteria; however, 13.9% of these (21 of 151 with morphologic evolution) evolved to FID within the first 6 months. Ninety-two cases suffered clinical aorta-related events (16.3%; mean follow-up range: 15–85 months; median: 52 months). Patients with TID had a similar risk of aorta-related events to those without ID (RR = 0.904; 95% CI, 0.335–2.440; P = 0.842; I2 = 42.5%), but lower than those with FID (RR = 0.299; 95% CI, 0.094–0.952; P = 0.041; I2 = 26.9%). Conclusions Tiny intimal disruption in type B IMH evolution is not related to an increased risk of complications and should not be considered an indication for invasive treatment. However, since 14% of TID evolve to FID within the first 6 months, close follow-up with imaging techniques is advisable.

Objective The aim is to present current clinical practice of thoracic aorta screening in a tertiary referral center. We identified how often imaging techniques were used for screening and established the value of transthoracic echocardiography (TTE) in comparison with computed tomography (CT) to detect aortic dilation. We also investigated which additional abnormalities of the heart, aorta or smaller arteries were discovered. Design All patients ≥15 years who visited our tertiary center in 2012-2016 for first thoracic aortic screening were retrospectively included. Diameters of the sinus of Valsalva (SoV) and maximum ascending aorta (AA) were compared between TTE and CT. The sensitivity and specificity of TTE to detect aortic dilation (≥40 mm) was assessed with CT as reference standard. Intracardiac abnormalities found with TTE and arterial abnormalities found with CT were identified. Results In total 349 patients (155 men, age 41 ± 15 years, 10% genetic mutation) were included. Screening was performed with TTE only in 35% and with TTE and CT in 65%. Patients who underwent TTE only were younger, had less often hypertension and less often a family history of aortic pathology. Although there was a good correlation between TTE and CT, the diameters measured with TTE were typically lower (SoV -1.0, 95%CI -6.6 to 4.7 and AA -0.4, 95%CI -6.5 to 5.8). Sensitivity of TTE for detecting aortic dilation was 61% (SoV) and 57% (AA) and specificity was 96% (SoV) and 100% (AA). Valve abnormalities, ventricular dilation or reduced ventricular function was found with TTE in 26 patients (7%). In 47 patients (13%) ascending aortic dilation was diagnosed and in 10 patients (4%) relevant peripheral arterial abnormalities were identified using CT. Conclusions Most often patients received both TTE and CT (65%). Since TTE showed a low sensitivity to detect aortic dilation, CT imaging is advised at least once in patients referred for thoracic aortic screening.

A 10-year-old boy seen in genetics clinic following a recent diagnosis of Marfan’s syndrome. The geneticist mentioned the use of prophylactic beta-blockers to reduce the risk of aortic dilatation. The boy’s mother is concerned about the prospect of taking daily medication from an early age and asks if this is necessary. ’Should beta-blockers (intervention) be commenced in all paediatric Marfan’s patients (patient) to prevent aortic dilation (outcome)? The Cochrane Library with the search terms ‘Marfan’s’ and ‘beta blockers’ yielded two reviews (one relevant) and three trials (two relevant). EMBASE (All Resources) (Ovid) — (Marfan’s and (beta blocker or beta blockade or beta-blocker or beta-blockers) and (children or adolescent or paediatrics or adolescents or kids or child or paeds)).af. Four hundred and sixty-five references were identified. Abstracts were screened for suitability. Studies in an exclusively adult population, those looking only at the biophysical properties of the aorta and those in animal models were excluded. This yielded 10 further papers. PubMed — (MeSH ((Marfan’s) AND (beta blocker OR beta blocker OR beta blockade OR beta-blocker)) AND (children OR adolescent OR paediatrics OR adolescents OR kids OR child OR paeds). Sixteen references …

The article is focused on a variety of aspects of aortic pathology in connective tissue dysplasias; approaches discussed to diagnostics and management of aortic dilation in dysplasias.

The primary goal of pre-participation cardiac evaluation in athletes is to identify individuals at high risk of sudden death but other cardiac abnormalities may be discovered. Athletes tend to have larger aortic dimensions than sedentary controls however it is unclear if this represents an aortopathy or is a normal physiological response to physical conditioning. To better understand the clinical implications of an enlarged aortic root in young athletes, Gati and colleagues1 compared aortic size and clinical outcomes in 3781 athletes with no evidence of a genetic aortopathy (mean age 19 years, 63% male) to 806 control subjects. This study found an enlarged aortic diameter in only 0.3% of athletes with no evidence of progressive enlargement over 5 years of follow-up. (figure 1) Figure 1 Absolute aortic root dimensions in athletes and controls. In an editorial, Jorstad and Groenink2 concur with the author’s conclusion that there appears to be little effect of physical activity on aortic size in young athletes without an underlying connective tissue disorder. The two other points they emphasise are that1 (1) reproducibility of aortic root measurements is not ideal and needs to be considered in patient follow-up and2 (2) pathological aortic dilation most often occurs in older individuals raising the concern that athletic activity as a teenager or young adult might affect aortic size decades later. They conclude that: ’While a …

Background Current treatment options and outcomes for acute uncomplicated thoracic Type-B aortic dissection (TBAD) remain unclear between medical management (MED) and thoracic endovascular aortic repair (TEVAR). In this study we aim to compare both strategies in terms of all-cause mortality, aortic dilation, and aortic rupture. Methods MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials were queried from January 1990 through March 2017. Only studies comparing TEVAR to MED for acute uncomplicated TBAD were included. Random-effects meta-analysis was used to pool outcomes across studies. Study outcomes included short (1 month), intermediate (1 year), and mid-term (2-5 year) all-cause mortality. Additional outcomes included aortic dilation and rupture at 1 year. Results A total of 1,960 patients (64.3 years; 75.8% male) were included from six studies (one prospective and five retrospective). No difference was observed in short-term (odd ratio [OR] 0.73 with 95% confidence interval [CI] 0.47 to 1.12, P = 0.15), intermediate (OR 0.99 with 95% CI 0.56 to 1.73, P = 0.96), or mid-term all-cause mortality (OR 1.12 with 95% CI 0.54 to 2.32, P = 0.75). No difference in aortic dilation with either modality was noted at 1-year (OR 1.11 with 95% CI 0.76 to 1.64, P = 0.59). TEVAR was associated with a significantly lower 1-year risk of aortic rupture (OR 2.49 with 95% CI 1.23 to 5.06, P = 0.01). Conclusion There were no short, intermediate, or mid-term differences in mortality between TEVAR or MED in patients with acute uncomplicated TBAD. Although the dilation rate was similar between both groups, TEVAR was associated with lower likelihood of aortic rupture at 1 year.