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The aortopulmonary window (APW) is a potential therapeutic target for a variety of conditions, including congenital heart disease and pulmonary hypertension. However, traditional methods for simulating the APW are often invasive and carry significant risks. This paper explores the potential of using 3D modelling in blender to develop a novel, noninvasive method for simulating the APW. We present a 3D model of the APW created in blender, which incorporates anatomical details such as the aorta, pulmonary valve, and the surrounding tissues. The model is then used to simulate the effects of different stimulation techniques, such as electrical stimulation and pressure waves. Our results suggest that 3D modelling in blender can be a valuable tool for developing and testing new methods for simulating the APW. The proposed method has several potential advantages over traditional methods. It is noninvasive, which avoids the risks associated with surgery. It is also highly versatile, as the stimulation parameters can be easily customized to the individual patient. Additionally, the 3D model can be used to visualize the effects of stimulation on the APW, which can provide valuable insights into the mechanisms of action. While further research is needed to validate the proposed method, our results suggest that 3D modelling in blender has the potential to revolutionize the treatment of conditions affecting the APW. [ABSTRACT FROM AUTHOR]

The article discusses a case of a 25-year-old primigravida with a fetus showing mirror-image dextrocardia and thoracic-abdominal discordance, detected through fetal echocardiography. Three-dimensional high-definition live tissue virtual dissection, including HD-live tissue rendering and 3D/4D spatiotemporal image correlation HD-live color rendering, was utilized to comprehensively analyze the complex cardiac morphology.

To describe prevalence, outcome, color Doppler, and HDlive Flow (Silhouette) with spatiotemporal image correlation (STIC) features of isolated tortuous ductus arteriosus in Japanese fetuses at 28–31 + 6 weeks of gestation. During a 37-month period, 3,150 fetal screenings were performed at 28–31 + 6 weeks of gestation. Isolated tortuous ductus arteriosus was evaluated using color Doppler and HDlive Flow (Silhouette) with STIC. Prevalence and outcomes were also assessed. There were 22/3,150 fetuses (0.7%) had isolated tortuous ductus arteriosus at 28–31 + 6 weeks of gestation. According to color Doppler and HDlive Flow findings, two types of isolated tortuous ductus arteriosus (Right-angled and Loop shapes) were classified. There were 20 Right-angled and 2 Loop shapes. One fetus with a Right-angled shape was associated with aortic tortuosity. HDlive Flow with STIC revealed spatial relationships of tortuous ductus arteriosus, aortic arch, and descending aorta. All fetuses with isolated tortuous ductus arteriosus had good neonatal outcomes. The incidence of isolated tortuous ductus arteriosus in Japanese fetuses was low compared with those in previous reports. HDlive Flow with STIC provides precise information for spatial recognition and differentiation of isolated tortuous ductus arteriosus, resulting in two type classifications. Isolated tortuous ductus arteriosus in a fetus may be a transient, benign finding in utero. [ABSTRACT FROM AUTHOR]

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A right aortic arch is present in 0.1% of the population and can occur in isolation or be associated with congenital heart disease.1 Moreover, the most common form of right aortic arch in adults is associated with an aberrant left subclavian artery.1 An aberrant left common carotid artery that originated from the ascending aorta with the right aorta is very rare. In this situation, carotid direct access was considered to avoid access challenge due to a large curve from the ascending aorta to the left common carotid artery.2 3 Here we demonstrate carotid artery direct access for intracranial stenting of a stroke patient with aberrant left common carotid artery and right aorta. Manual compression with a long time under general anesthesia to avoid post-procedural puncture site hematoma is recommended (video 1). neurintsurg;16/9/955/V1F1V1Video 1 Carotid artery direct access., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Anomalous origin of a pulmonary artery branch from the aorta is a rare congenital anomaly in which one of the pulmonary arteries arises from the aorta. These patients require early surgery to prevent development of severe irreversible pulmonary arterial hypertension. Multiple techniques have been described for repair of this condition. In this report, we describe a different technique compared with previously described procedures and discuss its advantages., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background. Nanotechnology developed multiple systems that may be involved in drug delivery to the target tissue, among them, gold nanoparticles (AuNPs) being considered potent and safe. Aims. The study aims to investigate oxidative stress and the modifications of the aorta wall after administration of gold nanoparticles, as the single component or functionalized with Cornus mas L. extract. Methods. Adult female rats received solutions by gavage, as follows: group C (control), saline solution; group I (citrate), trisodium citrate dehydrate; group II (AuNPs), gold nanoparticles solution; and group III (AuNPsCM) solution of gold nanoparticles functionalized with Cornus mas L. extract. Oxidative stress parameters were investigated in the serum and aorta, biochemical parameters from blood, diameters and blood flow velocity were measured and transmission electron microscopy (TEM) was performed on ascending aorta ring sections. Results. LDL was significantly reduced; MDA decreased significantly in serum; in the aorta, endothelin 1 and TNF-α were significantly increased in AuNPs and AuNPsCM groups, iNOS increased and MDA decreased in all treated groups, compared to the control group. The aorta diameters significantly decreased, consequently the blood flow increased significantly in the treated groups, in comparison with the control group. TEM investigation showed intima and media alterations in groups that received citrate and AuNPs, and only intima modifications in the AuNPsCM group. Conclusions. The aorta wall presented oxidative stress and histological modifications in all treated rats. [ABSTRACT FROM AUTHOR]

Purpose: Dilated aortic root and ascending aorta (AAO) with progressive aortic regurgitation is a wellknown sequela after arterial switch operation (ASO) in adults with transposition of the great arteries (TGA). We aimed to quantitatively assess aortic flow profiles in adults with TGA after ASO (Jatene procedure with LeCompte maneuver) using echo planar imaging (EPI) 4D flow MRI. Methods: Prospectively, 9 consecutive adults (30.2 ± 6.6 years) after ASO (Jatene operation with LeCompte technique), 13 consecutive adults (34.3 ± 7.2 years) after the atrial switch operation with Senning procedure, and 8 age-matched control patients, who underwent turbo field echo (TFE) EPI 4D flow MRI (average scan time of approximately 4 min), were enrolled. Results: TGA after ASO showed a markedly dilated sinus of Valsalva, compared to TGA after atrial switch operation (26.6. ± 4.9 vs. 18.6. ± 1.5 mm/cm2). Vorticity, helicity, wall share stress (WSS), and energy loss (EL) in the aortic root and the AAO in TGA were greater than in the controls. Vorticity, helicity, WSS, and EL in the aortic root and the AAO were also greater in TGA after ASO than after atrial switch operation. More acute aortic arch angle correlated with greater vorticity of the aortic root, and the significant diameter ratio of the sinus of Valsalva and the AAO was relevant to greater vorticity, helicity, and EL in TGA after ASO. Conclusion: A non-physiological blood flow pattern of the aortic root was identified in TGA adults after the ASO (Jatene procedure with LeCompte maneuver). Missing spiral looping of the great arteries and the unique structure after the Jatene procedure may play an adjunctive role in promoting aortopathy. The evaluation of aortic flow profile using EPI 4D flow MRI may be useful for risk stratification for aortopathy in this population. [ABSTRACT FROM AUTHOR]

Introduction: Prenatal recognition of dilated aortic root is extremely rare and there are significant challenges in counselling these patients. The primary aim of this case series is to describe the prevalence, associations and outcome of dilated ascending aorta diagnosed during fetal life.Methods: This is a retrospective cohort study from two tertiary fetal cardiology centres. Dilated ascending aorta was defined as gestation-specific standard deviation > 1.96 at some point during gestation.Results: Sixteen infants were live born and underwent postnatal echocardiography. Prenatally suspected bicuspid aortic valve (BAV) (n = 6) was confirmed in 5 cases (83%) postnatally. Thirteen children have been followed up for a period of minimum one year. No connective tissue disease was found.Conclusions: Prenatal dilated ascending aorta is a rare finding (0.06%). It is associated with BAV in 37% of cases and extracardiac abnormalities in 15.7%. Nuchal translucency measurement was >3.5 in 13% of cases. Connective tissue disease was not diagnosed postnatally. This is the largest prenatal cohort with dilated ascending aorta and postnatal outcomes to date. We showed a postnatal persistence of ascending aortic dilatation in 43% of babies. In the absence of extra-cardiac abnormalities, medium term outcome appears good but postnatal surveillance of aortic dilation is required. [ABSTRACT FROM AUTHOR]

This study aimed to describe the safety and efficacy of covered stents in patients with coarctation of aorta (CoA) for immediate and long-term follow-up. Covered stents are increasingly being used in (re)CoA, mainly to reduce the risk of aortic wall injuries (AWI). However, limited data are available on intermediate and long-term outcome. In 89 patients (67.4% male) with a mean age of 23.9 ± 15.8 (min max range 5.1–71.6) years were 102 covered stents implanted (January 2003 – December 2017). Short-term pre/post-implant hemodynamics and angiographic data were reported. Changes in blood pressure, the use of antihypertensive drugs and complications were recorded during follow-up. The procedural success rate was 100%. The mean invasive ascending-to-descending aorta systolic gradient under general anaesthesia decreased from 25 ± 16 mmHg to 4 ± 7 mmHg (p < 0.001). After a mean follow-up time of 6.6 ± 3.7 years, there was a persistent improvement of the mean systolic blood pressure gradient between right arm and leg (−7 ± 18 vs 38 ± 24 mmHg; p < 0.001). A larger proportion of patients required antihypertensive medication (33.7% vs 50.0%, p = 0.017) and needed ≥ 2 drugs (20.2% vs 27.4%, p = 0.066) to control blood pressure. Long-term adverse events were found in 4.5% of patients [covered stent fracture (n = 3), aneurysm formation (n = 2)]. Covered stent implantation for CoA is highly successful, safe and results in a persistent hemodynamic improvement in the immediate and long-term outcome. Lifelong follow-up with additional antihypertensive drug treatment is mandatory to maintain favourable hemodynamic results after stenting. Long-term follow-up data on covered stents in patients with coarctation of the aorta are scarce. A cohort of 89 patients was reviewed. The procedural implantation success rate was 100%. The invasive gradient decreased from 25 ± 16 mmHg to 4 ± 7 mmHg (p < 0.001). After follow-up of 6.6 ± 3.7 years, there was a persistent improvement of the clinical systolic blood pressure gradient (−7 ± 18 vs 38 ± 24 mmHg; p < 0.001). However, a larger proportion of patients required antihypertensive medication (33.7% vs 50.0%, p = 0.017). Covered stent implantation results in favourable hemodynamic effects, but lifelong follow-up with additional antihypertensive drug treatment is mandatory to maintain these results. [ABSTRACT FROM AUTHOR]

Arterial tortuosity syndrome is an extremely rare hereditary connective tissue disorder. We present a case of an incidentally diagnosed aneurysm of the aortic root and the ascending aorta caused by arterial tortuosity syndrome, which was confirmed genetically. The aneurysm was repaired surgically. One year after the procedure, there was no further dilation of the aorta or formation of new aneurysms.

Dual anterior interventricular artery is a rare type of CHD. We reported a fifteen-year-old girl who underwent CT angiography that demonstrated one anterior interventricular artery from aorta and another from pulmonary artery.

We herein present an exceptionally rare instance of a unicommissural unicuspid aortic valve (UAV) manifesting with an aneurysmal ascending aorta complicated by a type A aortic dissection ., (© 2024 Wiley Periodicals LLC.)

Background: Prenatal diagnosis of Berry syndrome, a rare combination of cardiac anomalies including aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), interrupted aortic arch (IAA), hypoplastic aortic arch, or coarctation of the aorta (COA), poses a significant challenge. Due to the rarity of the disease, and the limited case reports available to features the complex malformation of Berry syndrome postpartum, this article introduces an innovative approach to visually showcase this unusual disease. The proposed method provides a comprehensive display of the structural deformities, offering valuable insights for clinical practitioners seeking to comprehend this condition., Case Presentation: In this report, we present a case where fetal echocardiography aided in diagnosing Berry syndrome, which was later confirmed through postpartum cardiovascular casting. Our experience highlights the importance of using the three-vessel view to diagnose APW and aortic origin of the right pulmonary artery. Additionally, obtaining true cross-sectional and sagittal views by continuously scanning from the three-vessel-trachea view to the long-axis view of the aortic arch is necessary to image IAA or coarctation of the aortic arch., Conclusions: Early and accurate prenatal diagnosis of Berry syndrome is feasible and our cardiovascular cast can perfectly display the microvascular morphology of the fetal heart, which may have great application prospects for postpartum diagnosis and teaching of complex cardiac abnormalities., (© 2024. The Author(s).)

Background Limited research has been conducted on the surgical management of the aortic valve in congenitally corrected transposition of great arteries (ccTGA) and to our knowledge there have been no reports on the treatment of bicuspid aortic regurgitation (AR) in ccTGA. We report on a ccTGA patient with bicuspid AR and systemic right ventricule (SRV) dysfunction who underwent transcatheter aortic valve replacement (TAVR). Case summary A 17-year-old male with a history of ccTGA and cerebral palsy diagnosed at birth presented with heart failure. During childhood, he did not experience any heart failure symptoms, however, secondary to progressive bicuspid AR he experienced worsening SRV dysfunction beginning at 15-year-old. Echocardiography showed reduced SRV ejection fraction and severe bicuspid AR. The heart team, including a cardiac surgeon and paediatric cardiologist, discussed the treatment strategies and decided to proceed with TAVR as surgical aortic valve replacement was deemed high risk. TAVR was performed with the 34 mm Evolut R (Medtronic, Minneapolis, MN, USA). Post-operative echocardiography showed severe paravalvular leak (PVL). Therefore, valve-in-valve TAVR using a 29 mm Edwards SAPIEN 3 (Edwards Lifesciences, Irvine, CA, USA) was performed on post-operative Day 2 for PVL reduction. Following second procedure, PVL was significantly improved. The patient was discharged in stable condition. Discussion This is the first case wherein TAVR was performed for bicuspid AR in a patient with ccTGA. With appropriate preparation and planning and a collaborative multi-disciplinary team approach, TAVR can be a treatment option for severe AR in patients with ccTGA at high risk for surgery. [ABSTRACT FROM AUTHOR]

Computed tomography angiography (CTA) is one of the most common vascular imaging modalities. However, for clinical use, it still requires laborious manual analysis. This study demonstrates the feasibility of a fully automated technology for the accurate detection and identification of several anatomical reference points (landmarks), commonly used in intravascular imaging. This technology uses two different approaches, specially designed for the detection of aortic root and supra-aortic and visceral branches. In order to adjust the parameters of the developed algorithms, a total of 33 computed tomography scans with different types of pathologies were selected. Furthermore, a total of 30 independently selected computed tomography scans were used to assess their performance. Accuracy was evaluated by comparing the locations of reference points manually marked by human experts with those that were automatically detected. For supra-aortic and visceral branches detection, average values of 91.8 % for recall and 98.8 % for precision were obtained. For aortic root detection, the average difference between the positions marked by the experts and those detected by the computer was 5.7 ± 7.3 mm. Finally, diameters and lengths of the aorta were measured at different locations related to the extracted landmarks. Those measurements agreed with the values reported by the literature. Graphical abstract Schematic description of the proposed algorithm. The input includes an already segmented aorta (left), there are two main sub-processes related to the detection of branches and roots (center), and the output includes the segmented original aorta with the branches and the detected landmarks superimposed (right). [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVE: Detecting and accurate determining of vascular embolism dimensions are considered as a challenging issue in CT imaging. Present study aimed to enhance the accuracy of vascular embolism volumetry in managing a proper diagnosis and treatment of the disease. METHODS: In this non experimental quantitative study, firstly we simulated coronary and aorta arteries with normal dimensions in a 4D extended NURBS based cardiac-torso phantom in a matrix sizes of 512×512 with slice thickness of 0.116 mm. Then, twenty five venous thromboembolisms with the diameters ranged 0.1-5 and 5-20 mm were created in the coronary arteries and aorta of the Phantom, respectively. The Medical Imaging Interaction Toolkit was used for localization and volume measurement of the produced venous thromboembolisms on the CT images. Finally, Accuracy of the measured data was compared with the simulated measures in the phantom. FINDINGS: The difference on measures with the software was obtained 0.03±0.021 for embolies less than 4.64 mm (0.1 ml), (r=0.67, p=0.02), and 0.02±0.008 mm for embolies greater than 4.64 mm (r=0.99, p<0.001). CONCLUSION: According to the acceptable volumetry accuracy of Medical Imaging Interaction Toolkit especially for volumes greater than 0.1 ml, may be used for an accurate thromboembolism and pulmonary embolism measurements. [ABSTRACT FROM AUTHOR]

Importance: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit., Objective: To deliver the first heart valve implant that grows., Design, Setting, and Participants: Case report from a pediatric referral center, with follow-up for more than 1 year. Participants were a recipient neonate with persistent truncus arteriosus and irreparable truncal valve dysfunction and a donor neonate with hypoxic-ischemic brain injury., Intervention: First-in-human transplant of the part of the heart containing the aortic and pulmonary valves., Main Outcomes and Measures: Transplanted valve growth and hemodynamic function., Results: Echocardiography demonstrated adaptive growth and excellent hemodynamic function of the partial heart transplant valves., Conclusions and Relevance: In this child, partial heart transplant delivered growing heart valve implants with a good outcome at age 1 year. Partial heart transplants may improve the treatment of neonates with irreparable heart valve dysfunction.

Anomalous origin of the pulmonary arteries from the ascending aorta is a rare, but severe clinical entity necessitating a scrupulous evaluation. Either the right or the left pulmonary arteries can arise directly from the ascending aorta while the other pulmonary artery retains its origin from the right ventricular outflow tract. Such a finding can be isolated or can coexist with several congenital heart lesions. Direct intrapericardial aortic origin, however, must be distinguished with origin through a persistently patent arterial duct. In the current era, clinical manifestations usually become evident in the newborn rather than during infancy, as used to be the case. They include respiratory distress or congestive heart failure due to increased pulmonary flow and poor feeding. The rate of survival has now increased due to early diagnosis and prompt surgical repair, should now be expected to be at least 95%. We have treated four neonates with this lesion over the past 7 years, all of whom survived surgical repair. Right ventricular systolic pressure was significantly decreased at follow-up. Our choice of treatment was to translocate the anomalous pulmonary artery in end-to-side fashion to the pulmonary trunk. Our aim in this report is to update an Italian experience in the diagnosis and treatment of anomalous direct origin of one pulmonary artery from the aorta, adding considerations on the lessons learned from our most recent review of the salient literature. [ABSTRACT FROM AUTHOR]

Abstract L-NAME-induced hypertension is commonly used to study endothelial dysfunction and related vascular effects. It has been reported that genetic deletion of A 1 adenosine receptor (AR) reduces blood pressure (BP) increases in mice and thus, suggesting the involvement of A 1 AR. Thus, we sought to determine whether A 1 AR-induced vascular responses were altered in this mouse model of hypertension. L-NAME (1 mg/ml) was given in the drinking water for 28 days to mice. The BP was monitored using non-invasive tail-cuff system. Muscle tension studies were performed using DMT for mesenteric arteries (MAs) and organ bath for aorta. Protein expression was analyzed by western blot. Significantly, higher systolic and mean arterial blood pressure was noted in L-NAME mice. In MAs, higher 2-Chloro-N6-cyclopentyladenosine (CCPA, selective A 1 AR agonist) induced contractions in hypertensive mice were observed. This enhanced contraction was inhibited by HET0016 (Cytochrome 450 4A inhibitor, 10 µM, 15 min). Contrary, 5′-(N-Ethylcarboxamido) adenosine (NECA, non-selective AR agonist) induced vascular responses were comparable in both groups. Pinacidil (K ATP channel opener) induced relaxation was significantly increased in hypertensive mice. In aorta, CCPA-induced contractions were enhanced and inhibited by HET0016 in hypertensive mice. Notably, NECA-induced contractions in aorta were enhanced in hypertensive mice. Higher expressions of A 1 AR and Cyp4A were noted in MAs of hypertensive mice. In addition, in aorta, higher A 1 AR and comparable Cyp4A levels were observed in hypertensive mice. A 1 AR-induced vascular contractions were enhanced in hypertensive mice aorta and MAs. Cyp4A plays a role in altered vascular responses in MAs. [ABSTRACT FROM AUTHOR]

The article offers information about Thoracic aortic aneurysm disease. Topics discussed include disease occurrence due to walls of a blood vessel weaken, causing it to enlarge or dilate; talks about cystic medial degeneration and weakness in aorta wall are cause of the aneurysm; and mentions Beta-blocker blood pressure medications as treatment of the disease.

The term "coronary artery anomalies" encompasses a large and heterogeneous group of disorders that may affect origin, intrinsic anatomy, course, location, and termination of the coronary arteries. With these different anatomies, presentation, symptoms, and outcomes are heterogeneous as well. While significant efforts are directed toward improving diagnosis and risk-stratification, best evidence-guided practices remain in evolution. Data about anesthetic management of patients with coronary anomalies are lacking as well. This review aims to provide the anesthesiologist with a better understanding of an important subgroup of coronary artery anomalies: anomalous aortic origin of a coronary artery. We will discuss classification, pathophysiology, incidence, evaluation, management, and anesthetic implications of this potentially fatal disease group. [ABSTRACT FROM AUTHOR]

Because it is not known how often and how uniformly exercise testing is used to "unmask" recurrent or persistent coarctation of the aorta, this study was designed to determine (a) the frequency with which exercise testing is used by the clinician to evaluate patients after coarctation repair, and (b) the hemodynamic measures obtained with exercise that are considered to be indicative of significant persistent or recurrent coarctation. Questionnaires were sent to 80 randomly selected pediatric cardiologists; 49 were returned completed. About half of the respondents performed exercise testing (ET) on all of their patients after coarctation repair and 75 % tested at least half. Those who supervised an exercise laboratory used ET for a significantly greater number of their patients. In descending order, rest arm-leg gradient (ALG), maximal exercise systolic blood pressure (MXBP), and postexercise ALG were considered the most important indicators of significant recoarctation. The majority of respondents made decisions based on data from the ET that were consistent with published guidelines. [ABSTRACT FROM AUTHOR]

Takayasu arteritis is a large vessel arteritis described in young females. It presents with stenotic lesions of the aorta and its branches, although aneurysmal presentation is rare. A 62-year-old woman with Takayasu arteritis was admitted with complaints of abdominal pain and bilious vomiting for two days. She had a history of stenting of the right renal, superior mesenteric, and celiac artery. Computed tomography revealed a superior mesenteric artery pseudoaneurysm. Surgery was decided and it was successfully repaired. In conclusion, although rare, aneurysms of the superior mesenteric artery should be kept in mind in patients with Takayasu arteritis. [ABSTRACT FROM AUTHOR]

Background: Anomalous aortic origin of the left coronary artery with intraseptal course is a rare coronary anomaly associated with an increased risk of myocardial ischaemia. The role and techniques for surgical intervention are evolving, with numerous novel surgical techniques for this challenging anatomy reported in the last 5 years. We report our single-centre experience with surgical repair of intraseptal anomalous left coronary artery in the paediatric population, including clinical presentation, evaluation, and short- to mid-term outcomes., Methods: All patients with coronary anomalies presenting to our institution undergo standardised clinical evaluation. Five patients aged 4 to 17 years underwent surgical intervention for intraseptal anomalous aortic origin of the left coronary artery between 2012 and 2022. Surgical techniques included coronary artery bypass grafting (n = 1), direct reimplantation with limited supra-arterial myotomy via right ventriculotomy (n = 1), and transconal supra-arterial myotomy with right ventricular outflow tract patch reconstruction (n = 3)., Results: All patients had evidence of haemodynamically significant coronary compression, and three had evidence of inducible myocardial ischaemia pre-operatively. There were no deaths or major complications. Median follow-up was 6.1 months (range 3.1-33.4 months). Patients who underwent supra-arterial myotomy (with or without reimplantation) had improved coronary flow and perfusion based on stress imaging and catheterisation data., Conclusions: Surgical approaches to intraseptal anomalous left coronary artery with evidence of myocardial ischaemia continue to evolve, with new techniques demonstrating promising improvement in coronary perfusion. Further studies are warranted to determine long-term outcomes and refine indications for repair.

An aorto-ventricular tunnel is a rare congenital cardiac defect, where a channel connects the lumen of the ascending aorta to the left or right ventricle. Four patients presented with an aorto-left ventricular tunnel over two decades at a median age of 8 months (range 0.1-10 months). Two patients (50%) had associated cardiac anomalies including hypoplastic left heart syndrome and left ventricular noncompaction/hypertrophic cardiomyopathy with aortic/pulmonary valve dysplasia in one patient each. Although traditionally surgical treatment has addressed this problem, management has evolved to transcatheter closure with excellent outcomes in appropriately selected patients at our national centre.

Turner syndrome (TS) is a common genetic disorder in females with high incidence of ascending aortic dilatation and even dissection occurring as early as in the second decade. Known risk factors (RF) are bicuspid aortic valves (BAV), coarctation of the aorta (CoA), and arterial hypertension. Since 10% of dissections occur in patients without RF, an intrinsic aortic wall abnormality has been postulated. This study aimed to investigate the elasticity of the ascending aorta as a surrogate marker of aortic wall texture. Forty-six pediatric patients with genetically proven TS were prospectively examined for the morphology of their aortic valve, and size and elasticity indices of the adjacent aorta. Cohorts of 46 female subjects with tricuspid aortic valves (TAV) and ten non-syndromic females with BAV were investigated as separate control groups. Comparison of healthy controls with TS patients revealed significantly deteriorated elasticity indices in those with TS. Furthermore, normalized aortic dimensions were greater in TS patients, but dilatations of the ascending aorta with z-score levels above two were restricted to those with BAV (14/46). Deteriorated elasticity indices were measured in TS patients, independent of aortic dilatation, BAV, and CoA, and were comparable to those of patients with isolated, non-syndromic BAVs. By measuring elasticity levels as a surrogate for aortic wall texture, we were able to gather evidence that TS presents with an intrinsic abnormality of the ascending aorta even in patients without concomitant BAV, CoA or dilatations as early as in childhood. [ABSTRACT FROM AUTHOR]

Abstract Abdominal aortic dissection (AAD) is very rare in the pediatric population in the absence of trauma, connective tissue disease, or infection. Our patient presented with an AAD causing aortoiliac occlusive disease (AIOD) and left popliteal arterial disease which is not described in the pediatric population. Presentation of the AIOD was classic in our pediatric patient though a distracting history of strained calf muscle during a sporting event delayed diagnosis by several months. Once the diagnosis was established, the interesting problem of managing this disease was met with possible risks of other vascular disease or even other organ disease. Further imaging and a genetic evaluation did not elucidate an etiology for our patient, only a suspicion. Post-surgical intervention was met with symptom resolution but warranted careful surveillance and follow-up in clinic by both genetics and vascular surgery. [ABSTRACT FROM AUTHOR]

Background/Aims: Vascular calcification and hypertension are intimately linked, and the progression of hypertension is closely correlated with endothelial dysfunction. However, the role of endothelial cells (ECs) in vascular calcification of hypertension remains unclear. Therefore, the present study explored the effects of ECs on calcification of smooth muscle cells (SMCs) from aortas of spontaneously hypertensive rats (SHR). Methods: Aortic ECs and SMCs were isolated from SHR and Wistar rats, respectively. The roles of ECs in the regulation of SMCs calcification were investigated by co-culture and conditioned culture model. Calcium deposition of SMCs was detected by von Kossa staining. Quantization of calcium content in SMCs was determined colorimetrically by the o-cresolphthalein complexone method. Alkaline phosphatase (ALP) activity was measured colorimetrically by p-nitrophenol. The expression levels of MMP-2, MMP-9 and the calcification-promoting proteins were analyzed by Western blot. Results: Calcium deposition, ALP activity and the expression levels of calcification-promoting proteins in SMCs of SHR were significantly higher than that cultured without ECs after 6 days of co-culture with ECs or conditioned culture with the medium of ECs, however, there were no statistical differences between SMCs of Wistar rats. MMP-2 and MMP-9 in co-cultured ECs from SHR were dramatically higher than that cultured without SMCs, nevertheless, there were no statistical differences between ECs from Wistar rats and between SMCs from SHR or Wistar rats. Moreover, SB-3CT, a specific inhibitor of gelatinases, decreased calcium content and the expression levels of calcification-promoting proteins in both co-cultured and conditionally cultured SMCs from SHR. Conclusion: ECs have the ability to promote calcification of aortic SMCs of SHR, and elevated expressions of MMP-2 and MMP-9 in ECs of SHR might facilitate the calcification of SMCs. [ABSTRACT FROM AUTHOR]

Summary: Activation of TRPV4 (transient receptor potential vanilloid 4) has been reported to result in endothelium‐dependent contraction in the aortae of hypertensive mice. This contraction involved increased cPLA2 (cytosolic phospholipase A2) activity. The mechanism by which TRPV4 regulates cPLA2 activity to induce contraction in hypertension, however, is unknown. Through measurements of arterial tension and protein level, we showed that high‐salt diet induced hypertension increases activity of PKC (protein kinase C) and ERK1/2 (extracellular signal‐regulated kinase 1/2). GSK1016790A, a TRPV4 agonist and ACh (acetylcholine) induced contractions were suppressed by Go6983, a PKC inhibitor and PD98059, an ERK1/2 inhibitor. TRPV4 activation increased activity of PKC and ERK1/2 in endothelial cells from hypertensive mice and this response was suppressed by HC067047, a TRPV4 inhibitor and BAPTA/AM, a Ca2+ chelator. PLA2 assay and western blotting showed that blocking of PKC or ERK1/2 inhibited TRPV4 or ACh‐induced cPLA2 activity. Enzyme immunoassay showed that GSK1016790A or ACh triggered the release of PGF2α (prostaglandin F2α) was reduced by inhibition of PKC or ERK1/2. These data further suggest Ca2+/PKC/ERK1/2 axis as a novel mechanism for TRPV4 in the activation of cPLA2 in hypertension. [ABSTRACT FROM AUTHOR]

Objectives: The role of aortic angulation in attenuating procedural success in balloon-expandable (BE) and self-expandable (SE) transcatheter aortic valve replacement (TAVR) has been controversial. Methods: We retrospectively assessed patients undergoing SE and BE TAVR who had an aortic angle measured on multidetector computed tomography at a single tertiary referral center. The primary outcome was device success, measured per the Valve Academic Research Consortium-2 criteria. Clinical outcomes at 30 days (including mortality) were also assessed. Results: A total of 251 patients were identified; 182 patients received a BE valve and 69 patients an SE valve. The median aortic angle was 46.8° (range 24.4–70°) in the BE group and 43.3° (range 20–71°) in the SE group. In multivariate logistic regression analysis, aortic angulation did not affect device success. Mortality at 30 days and 12 months and postprocedural clinical outcomes were similarly not associated with aortic angulation. Conclusion: In this cohort of patients undergoing BE and SE TAVR over a wide range of aortic angles, we found no associations between angle and device success or any other clinical metrics. Increased aortic angulation does not adversely affect outcomes in BE or SE TAVR. [ABSTRACT FROM AUTHOR]

Purpose: We evaluated anatomical characteristics and clinical significance of left vertebral artery (VA) originating from aortic arch (AA) by computed tomography (CT) angiography.Methods: CT angiography was performed in 3460 patients between March 01, 2014 and November 30, 2015. We examined course of prevertebral VA (PVVA) segment and level of entry into the cervical vertebra transverse foramen (CVTF) of left VA originated from AA.Results: One hundred fifty-three of 3460 patients had left VA originated from AA. Six of 153 patients had dual origin of VA. Entry level to CVTF of 156 left VAs in 153 cases ranged from C3 to C6. Entry level to CVTF of 156 right VAs in 153 cases ranged from C3 to C7. One hundred fifty-six right PVVA segments positioned in longus colli muscle lateral side in 112 VAs, longus colli muscle anterior surface near longus colli muscle lateral margin in 41 VAs, and unknown location in three VAs. One hundred fifty-six left PVVA segments positioned in anterior surface of longus colli muscle midline in 5 cases, anterior surface of longus colli muscle near longus colli lateral margin in 138 cases, longus colli muscle lateral side in 12 cases, and anterior surface of anterior scalene muscle midline in one case.Conclusions: Left VA may arise from the AA. If a long PVVA segment entering higher CVTF is present, operator can perform anterior cervical surgery via contralateral approach for avoidance of VA injury. [ABSTRACT FROM AUTHOR]

Purpose: The median sacral artery (MSA) is the termination of the dorsal aorta, which undergoes a complex regression and remodeling process during embryo and fetal development. The MSA contributes to the pelvic vascularization and may be injured during pelvic surgery. The embryological steps of MSA development, anastomosis formation and anatomical variations are linked, but not fully understood.Methods: The pelvic vascularization and more precisely the MSA of a human fetus at 22 weeks of gestation (GW) were studied using micro-CT imaging. Image treatment included arterial segmentations and 3D visualization.Results: At 22 GW, the MSA was a well-developed straight artery in front of the sacrum and was longer than the abdominal aorta. Anastomoses between the MSA and the internal pudendal arteries and the superior rectal artery were detected. No evidence was found for the existence of a coccygeal glomus with arteriovenous anastomosis.Conclusions: Micro-CT imaging and 3D visualization helped us understand the MSA central role in pelvic vascularization through the ilio-aortic anastomotic system. It is essential to know this anastomotic network to treat pathological conditions, such as sacrococcygeal teratomas and parasitic ischiopagus twins (for instance, fetus in fetu and twin-reversed arterial perfusion sequence). [ABSTRACT FROM AUTHOR]

The time course of angiotensin-converting enzyme activity in the rat aorta after fractionated exposure to ionizing radiation and the effects of dihydroquercetin and fucoidin on this parameter were studied. Male Wistar rats were exposed to single or repeated (fractionated) X-ray radiation in a dose of 2.5 Gy at 200 kV. Activity of angiotensin-converting enzyme in aorta segments was evaluated 2 h after the last exposure by hydrolysis of hippuryl-histidineleucin substrate. Enzyme activity in the rat aorta was higher than normally after all the studied doses of fractionated exposure (2.5 Gy per fraction) with the maximum effect after the total dose of 7.5 Gy (3 fractions). Fucoidin, a blocker of endothelium receptors realizing the leukocyte adhesion to the endothelium, and flavonoid dihydroquercetin inhibiting expression of adhesion molecules in the endothelium abolished the increase in activity of angiotensinconverting enzyme in the rat aorta after single exposure; moreover, dihydroquercetin reduced significantly the effect of fractionated exposure. These data indicate that leukocyte adhesion to the endothelium is an important factor contributing to the increase of angiotensin-converting enzyme activity in the aorta. [ABSTRACT FROM AUTHOR]

Introduction Turner syndrome (TS) is a genetic disorder characterized by the (partial) absence or a structural aberration of the second sex chromosome and is associated with a variety of phenotypes with specific physical features and cardio-aortic malformations. The objective of this study was to gain a better insight into the differences in dysmorphic features between girls and women with TS and to explore the association between these features, karyotype and cardio-aortic malformations. Methods This prospective study investigated 14 dysmorphic features of TS girls and women using a checklist. Three major phenotypic patterns were recognized (severe phenotype, lymphatic phenotype and skeletal phenotype). Patient data including karyotype and cardio-aortic malformations (bicuspid aortic valve (BAV) and aortic coarctation (COA)) were collected. Associations between the prevalence of dysmorphic features, karyotype and cardio-aortic malformations were analysed using chi 2 -test and odds ratios. Results A total of 202 patients (84 girls and 118 women) were analysed prospectively. Differences in prevalence of dysmorphic features were found between girls and women. A strong association was found between monosomy 45,X and the phenotypic patterns. Furthermore, an association was found between COA and lymphatic phenotype, but no association was found between karyotype and cardio-aortic malformations. Conclusion This study uncovered a difference in dysmorphic features between girls and women. Monosomy 45,X is associated with a more severe phenotype, lymphatic phenotype and skeletal phenotype. All patients with TS should be screened for cardio-aortic malformations, because in contrast to previous reports, karyotype and cardio-aortic malformations showed no significant association. [ABSTRACT FROM AUTHOR]

Introduction: Aerobic exercise has a favorable effect on systemic vascular function, reducing both central (large elastic artery) and peripheral (smaller muscular artery) stiffness. The effects of resistance exercise (RE) on arterial stiffness are more complex. Acute RE increases central artery stiffness while decreasing peripheral stiffness. To date, the majority of studies have been performed in predominantly male participants.Purpose: To examine the effect of acute RE on central and peripheral arterial stiffnesses in women, a secondary purpose was to explore the influence of cyclic changes in estrogen status across the menstrual cycle on the arterial response to acute RE.Methods: 18 healthy women [28 ± 7 years, body mass index (BMI) 22.6 ± 2.9 kg/m2] completed an acute RE bout during the early follicular and the early luteal phase of their menstrual cycle. Salivary 17β-Estradiol concentration was measured during each phase, using a passive drool technique. Pulse-wave velocity (PWV) was obtained from the carotid-femoral and carotid-radial pulse sites to measure central and peripheral stiffness, respectively, using applanation tonometry. PWV was measured at rest, immediately, 10, 20, and 30 min post-RE.Results: 17β-Estradiol concentration was significantly lower in the early follicular vs. the early luteal phase of the menstrual cycle (1.78 ± 0.51 vs. 2.40 ± 0.26 pg/ml, p = 0.01). Central PWV significantly increased (p < 0.05) and peripheral PWV significantly decreased (p < 0.05) post-RE in both the early follicular and early luteal phases. No phase-by-time interaction was detected for either vascular segment (p > 0.05).Conclusion: Women experience increases in central arterial stiffness and reductions in peripheral arterial stiffness following acute RE. Menstrual cycle phase may not influence changes in arterial stiffness in response to acute RE. [ABSTRACT FROM AUTHOR]

Circumflex aorta and double aortic arch are two forms of rare vascular rings. We present a case of an infant who was diagnosed with circumflex aorta and double aortic arch, and describe the surgical management of this rare anomaly. [ABSTRACT FROM AUTHOR]

Detection rates of congenital cardiac malformations have traditionally remained low. The NHS Fetal Anomaly Screening Programme (FASP) aims to increase these detection rates for various reasons, including influencing perinatal management and aiding parental decision making. The inclusion of the three vessel view and trachea (3VT) view in 2015 aimed to improve detection rates of arch abnormalities in particular. This study evaluated the early impact of the new initiative at one NHS Trust. Departmental screen-positive rates were compared for a full year before and after implementation. Referrals to, and opinions of, the foetal medicine unit (FMU) were assessed; as were undetected congenital heart defects for the two time periods. Compared with the pre-implementation (pre-3VT) period, the number of completed anomaly scans performed after implementation (post-3VT) increased by 3% and the number of FMU referrals increased by 625%. Departmental screen-positive rates for cardiac abnormalities increased from 40% (pre-3VT) to 91% (post-3VT). Over half (52%) of the FMU referrals were made due to a suspected abnormal 3VT view. Early evaluation of 3VT implementation at this NHS Trust indicates that it has been a success. Departmental screen-positive rates for congenital cardiac malformations have risen. However, this performance has come at a cost: Some abnormalities now being detected, such as loose vascular ring and PLSVC, are frequently asymptomatic and likely to be clinically insignificant. The implementation of 3VT achieves the aims of FASP but may begin to exceed what is expected from a low risk population screening programme. [ABSTRACT FROM AUTHOR]

Objectives: Stent implantation for the treatment of aortic coarctation has become a standard approach for the management of older children and adults. Criteria for optimal stent design and construction remain undefined. This study used computational modelling to compare the performance of two generations of the Cheatham-Platinum stent (NuMED, Hopkinton, NY, USA) deployed in aortic coarctation using finite element analysis. Design: Three-dimensional models of both stents, reverse engineered from microCT scans, were implanted in the aortic model of one representative patient. They were virtually expanded in the vessel with a 16mm balloon and a pressure of 2 atm. Results: The conventional stent foreshortened to 96.5% of its initial length, whereas the new stent to 99.2% of its initial length. Diameters in 15 slices across the conventional stent were 11.6-15mm (median 14.2 mm) and slightly higher across the new stent: 10.7-15.3mm (median 14.5 mm) (p= 0.021). Apposition to the vessel wall was similar: conventional stent 31.1% and new stent 28.6% of total stent area. Conclusions: The new design Cheatham-Platinum stent showed similar deployment results compared to the conventional design. The new stent design showed slightly higher expansion, using the same delivery balloon. Patient-specific computational models can be used for virtual implantation of new aortic stents and promise to inform subsequent in vivo trials. [ABSTRACT FROM AUTHOR]

Objective: To report clinical and procedural characteristics of twelve patients who received a covered stent for the treatment of aortic coarctation and concurrent patent ductus arteriosus (PDA). Methods: A single center database was retrospectively evaluated to obtain data of patients with combined aortic coarctation and PDA. We selected patients in whom a covered stent was used for the treatment of both pathologies. The stent length was chosen so as to cover the entire length of the lesion from healthy to healthy tissue and also cover the ampulla of PDA. Results: The median age of the patients was 15 (range, 6.5-35) years. The diameter of the coarctated segment increased from a median of 8.4 (range, 2.6-10.8) mm to 16 (range, 9-24) mm (p<0.005), whereas the pressure gradient decreased from a median of 43 (range, 10-71) mm Hg to 0 (range, 0-8) mm Hg (p<0.005). Fourteen covered stents were used for 12 patients. Following deployment, seven stents were flared with larger and low-pressure balloons because of the gap between the distal end of the stent and the poststenotic dilated segment of the aorta, which caused residual PDA shunts and/or instability of the stent. After the procedure, no residual PDA shunt was present in any patient. Conclusion: To the best of our knowledge, this study includes the largest series of patients reported in literature in whom covered CP stents were used for simultaneous percutaneous treatment of coarctation and PDA. The procedure was successful and stable results were obtained during follow-up in all cases. [ABSTRACT FROM AUTHOR]

Changes in the structural components of aortic tissues have been shown to play a significant role in the pathogenesis of aortic degeneration. Therefore, reliable stress analyses require a suitable and meaningful constitutive model that captures micro-structural changes. As recent data show, in-plane and out-of-plane collagen fiber dispersions vary significantly between healthy and aneurysmatic aortic walls. The aim of this study is to computationally investigate the influence of fiber dispersion on the mechanical response of aortic tissues in health and disease. In particular, the influence of three different fiber dispersions is studied: (i) non-rotationally symmetric dispersion, the most realistic assumption for aortic tissues; (ii) transversely isotropic dispersion, a special case; (iii) perfectly aligned fibers (no dispersion in either plane), another special case. Explicit expressions for the stress and elasticity tensors as needed for the implementation in a finite element code are provided. Three representative numerical examples are studied: planar biaxial extension, inflation of residually stressed and pre-stretched aortic segments and inflation of an idealized abdominal aortic aneurysm (AAA) geometry. For the AAA geometry the case of isotropic dispersion is additionally analyzed. Documented structural and mechanical parameters are taken from human aortas (healthy media/adventitia and AAA). The influence of fiber dispersions upon magnitudes and distributions of stresses and deformations are presented and analyzed. Stresses vary significantly, especially in the AAA case, where material stiffening is significantly influenced by fiber dispersion. The results highlight the need to incorporate the structural differences into finite element simulations to obtain more accurate stress predictions. Additionally, results show the capability of one constitutive model to represent different scenarios of aortic micro-structures allowing future studies of collagen reorientation during disease progression. [ABSTRACT FROM AUTHOR]

Background: Left nonrecurrent laryngeal nerve (LNRLN) is an extremely rare anatomic variant. The development of such anatomic variation requires the regression of both the fourth (aortic arch) and sixth (ductus arteriosus, DA) arches on the left side. Preoperative prediction of this variant is difficult but might reduce risk of nerve injury. Methods: A 34-year-old female was indicated for thyroidectomy for a 2.4 cm follicular neoplasm and Graves' disease. Due to a positive medical history of 22q11.2 microdeletion and unexplained left vocal cord paralysis, a preoperative chest computed tomography (CT) scan was obtained and revealed a right-sided aorta (RSA) and aberrant left subclavian artery (ALSA) without Kommerell's diverticulum. A left-sided NRLN was then highly suspected. Results: Thyroidectomy was performed under general anesthesia with the utilization of intraoperative laryngeal nerve monitoring. A LNRLN was confirmed intraoperatively. Conclusions: Right-sided aorta and ALSA indicate embryologic regression of the left fourth primitive aortic arch. The absence of Kommerell's diverticulum at the origin of the ALSA indicates the lack of high-pressure blood flow from the pulmonary artery to the ALSA through the ductus arteriosus during embryogenesis, suggesting the embryologic regression of the left sixth primitive aortic arch. The presence of all 3 radiologic features thus highly suggests the possibility of a LNRLN. [ABSTRACT FROM AUTHOR]

Background and aims Increased volume of visceral adipose tissue is associated with worsening of cardiovascular disease risk factors that contribute to aortic dilatation. We investigated the effects of visceral fat index (VFI) and VFI/percentage body fat (PBF) ratio on proximal aortic size and proximal aortic dilatation (PAD), to assess whether excess visceral fat deposition is an independent risk factor for PAD. Methods 738 participants aged 35 years or more were included in this cross-sectional survey. The sizes of aortic valve annulus (AVA), sinuses of Valsalva (SV), sinotubular junction (STJ), and ascending aorta (AscAo) were measured by transthoracic ultrasound. Multivariate linear regression, binary logistic regression, Bayesian linear regression, and receiver operating characteristic curves were performed to clarify the effects of VFI and VFI/PBF ratio on PAD. Results There were 78 participants (10.6%) with PAD. VFI and VFI/PBF ratio in the population with PAD was significantly increased, compared to the population without PAD ( p < 0.001). However, PBF was not significantly different between the two populations. VFI/PBF ratio was positively associated with sizes of AVA, SV, STJ, and AscAo ( p < 0.05), and was independently related to PAD ( p < 0.05). A 1-SD increment in VFI/PBF ratio was associated with 13.35-fold increased risk of PAD (odds ratio: 13.35, p < 0.05). Conclusions VFI/PBF ratio is independently associated with PAD. An increased proportion of visceral fat may contribute to PAD. VFI/PBF ratio calculation may be used for the preliminary identification of individuals at high risk of PAD in the Chinese population. [ABSTRACT FROM AUTHOR]

During routine dissection a rare type of branching pattern of the arch of aorta was seen. The left common carotid artery was originating from the brachiocephalic trunk instead of the arch of aorta. This kind of anomaly occurs due to abnormal development of the arch of aorta. The surgeons while operating on the neck region have to keep in mind about such type of anomaly. Also it is important for interventional radiologists who do carotid angiograms. [ABSTRACT FROM AUTHOR]

The basic theme of this work is to identify the optimal measurement locations for pressure and flow in the systemic circulation to detect aortic stenoses and aneurysms in early stages of a disease. For this purpose, a linear elastic lumped parameter model of the fluid dynamical simulator, major arterial cardiovascular simulator (MACSim), is considered and global sensitivity analysis is applied to identify the better measurement locations for pressure and flow in the systemic circulation. The obtained results of sensitivity analysis provide insight that enable the experimentalists to optimize their experimental setups for detecting aortic stenoses and aneurysms using parameter estimation process. From the results, it is observed that the stenosis in the thoracic aorta can be identified from both pressure and flow at the location itself, nearby nodes, aorta ascendens, arcus aorta, arteria subclavia and arteria axillaris. On the other hand, the preferable measurement locations for abdominal aneurysms are locations themselves, nearby nodes and left/right leg of the body. [ABSTRACT FROM AUTHOR]