Sinus Of Valsalva Aneurysm Research Articles

A Unique Case of Giant Aneurysm of Sinus of Valsalva

Abstract Sinus of Valsalva aneurysms (SOVA) arises most commonly from the right coronary sinus of the aorta. It causes aortic sinus dilatation above the annulus and occurs due to the weakness of the elastic lamina. In our case, a 63-year-old man came with complaints of insidious-onset and progressive exertional dyspnea and fatigue for the last 6 months. He was in NYHA functional class III. On examination, right-sided heart failure signs were noted. He was treated with antifailure medications and stabilized. Two-dimensional echocardiogram and cardiac computed tomography were done and a diagnosis was made.

Aortic Root Dilatation in Children With End-Stage Kidney Disease on Regular Hemodialysis and After Kidney Transplantation.

Cardiovascular disease (CVD) is the major cause of mortality in end-stage kidney disease (ESKD) patients. Aortic root dilatation (ARD) was recently recognized as a cardiovascular (CV) sequela in these patients. This study aims to evaluate the prevalence, risk factors, and progression of ARD in children with ESKD on regular hemodialysis (HD) and after kidney transplantation (KT). Seventy children in HD (HD group) and 80 pediatric kidney transplant recipients (KTR) (KT group) were included. The echocardiographic assessment was done twice at intervals of more than 6 months (6-14 months) except for 3 patients who died before the second assessment. ARD's overall prevalence was higher in HD than in KT groups (71.4% and 40%). ARD was more prominent in patients with increased duration of ESKD, EDW, normalized IDWG%, and normalized UF vol%. In the KTR group, underweight was more prevalent among the ARD group than the non-ARD group. Patients with ARD had higher BP than patients without ARD. Most of the patients with ARD were on classic triple immunosuppressive therapy. ARD patients of both groups had lower HB, higher PLTS, Ph, Ca × Ph levels, and lower e-GFR than non-ARD patients. ARD is prevalent among ESKD children, with furthermore prevalence in HD than in KT patients. Younger age, lower BMI Z-scores, hypertension (HTN), and increased Ca × Ph are risk factors for ARD. Aortic root measures and ARD frequency decrease on ≥ 6-month follow-up of patients.

Quadricuspid aortic valve in an asymptomatic young adult: a case report

Introduction: Quadricuspid aortic valve (QAV) is a rare congenital heart disease, ranking behind bicuspid and unicuspid aortic valves in terms of the incidence of congenital aortic valve abnormalities. When symptoms are present, they are typically related to aortic regurgitation, manifesting as shortness of breath, nocturnal dyspnea, and palpitations, or aortic stenosis, which presents with exertional dyspnea, angina, or syncope. Case presentation: We present the case of an asymptomatic male, diagnosed with a QAV during a routine examination. Despite the absence of clinical symptoms, a thorough general physical examination, including cardiac auscultation, revealed signs suggestive of valvular abnormality. The diagnosis was confirmed via transthoracic echocardiography. Clinical discussion: For QAV, the diagnostic process typically includes transthoracic echocardiography, which allows for initial confirmation of the valve morphology. In addition, computed tomography (CT) coronary angiography provides detailed information on valvular morphology and helps identify any associated coronary stenosis. In cases of QAV, a CT aortogram is also crucial to assess potential aortic root dilation, a known complication of this congenital anomaly. Conclusion: This case emphasizes the importance of routine physical examination in diagnosing rare congenital heart conditions like QAV, even in asymptomatic individuals. Early diagnosis and appropriate imaging are essential for timely management, particularly in preventing the progression of associated complications such as aortic regurgitation or root dilation.

Interventricular septal dissection in a patient with atrial septal defect and pulmonary hypertension

Background: Interventricular septal dissection (IVSD) is a sporadic disorder. Previous literature has reported cases of IVSD caused by sinus valsalva aneurysms, post-myocardial infarction patients, congenital abnormalities, or post-thoracic trauma. However, no prior literature has reported IVSD cases caused by an atrial septal defect (ASD) accompanied by pulmonary hypertension. This case report aims to initiate the discussion of recognizing the association between ASD-PH and IVSD. Case presentation: A 48-year-old female with ASD was brought to Dr. Sardjito General Hospital with complaints of fatigue. In 2013, the patient experienced attacks of hemoptysis, which was then revealed to be caused by an increase in pulmonary pressure. Transthoracic echocardiography (TTE) examination results concluded that the patient had a high probability of pulmonary hypertension (PH), secundum atrial septal defect (ASD), which was 22-25 mm in diameter, a bidirectional shunt, and mid-apical interventricular septum dissection with flow. The patient underwent a right heart catheterization examination on September 3rd, 2015, at Dr. Sardjito General Hospital and was confirmed with pulmonary hypertension. The patient underwent computed topography (CT) scan with contrast on March, 2024, for evaluation, where we found a rupture of the interventricular septal muscle. Conclusion: The incidence of interventricular septal dissection is rare. Various causes are suspected, such as sinus of Valsalva rupture, post-myocardial infarction events, or associated congenital abnormalities. From this report, we present an IVSD case that is concurrent with ASD-PH. ASD and pulmonary hypertension, through combination of myocardial injury and hypertension, can cause interventricular septal dissection. Keywords: interventricular septal dissection, atrial septal defect, pulmonary hypertension, case report

Total autologous reconstruction of the right and left ventricular outflow tracts: the case for the modified Ross-Ozaki procedure.

The Ross procedure continues to be the best procedure to address unrepairable aortic valve pathology, especially in young adults. The Achilles heel of this procedure has been aortic root dilation and the potential need for a reoperation that may be associated with slightly increased risks in addition to the need for intervention on the pulmonary outflow tract. Modifying the Ross procedure by autograft inclusion inside a Dacron graft seems to have the potential advantage of stabilizing the autograft diameter, which may be associated with improved durability and decrease the need for future intervention. Although the long-term data are satisfactory, the pulmonary homografts are costly and have limited availability, so the need for alternate options for reconstruction of the right ventricular outflow tract exists. Utilization of the autologous pericardium in the reconstruction of neo-pulmonary leaflets may be considered an alternative to other pulmonary valve reconstruction options. We present a few different modifications to the Ross procedure that may have the potential of being more reproducible and more cost effective, especially in areas of the world where homografts and bioprosthetic valves are not readily available.

Aortic regurgitation in ankylosing spondylitis-an echocardiography follow-up study.

To investigate the long-term course of aortic regurgitation (AR) and the width of the proximal ascending aorta (PAA) in patients with ankylosing spondylitis (AS). This is a follow-up cohort study of patients with AS examined with echocardiography at inclusion (2009 to 2011). Out of the initial 187, a subgroup of 52 patients (54% men, mean age 62years) was selected for follow-up based on presence/absence of AR at baseline; 26 with AR (18 mild, 7 moderate, 1 severe) and 26 age/sex-matched without AR. These patients were re-examined with echocardiography in 2014 by an independent observer. Severity of AR and PAA diameter wereassessed. Related samples Wilcoxon signed rank and Mann-Whitney U tests were used to analyze the change (Δ) in PAA diameter. Regarding the 26 patients with AR at baseline, two had an aggravated grade, 16 an unchanged grade, and eight a less severe AR versus baseline. Two of the 26 patients with no AR at baseline had a mild grade of AR at follow-up. The mean (SD) ΔPAA diameter was 0 (3) mm, and no statistically significant ΔPAA diameter was found overall or in analyses stratified by sex and baseline presence of AR. Most patients with AS had an unchanged grade of AR and PAA diameter at follow-up 3 to 5years after the initial echocardiography. These findings suggest that the average progress of AR in patients with AS is slow and that progression of PAA dilatation seems rare. Key points • Aortic regurgitation (AR) is not uncommon in patients with ankylosing spondylitis (AS) and caused by aortic root dilatation and/or cusp fibrosis/retraction, but little is known about its course. • According to this repeated echocardiography study in median 4.3years after the baseline evaluation, the majority of patients had no progress of AR or increase in the proximal ascending aorta diameter. • AR in AS is rarely rapidly progressive.

Z-score evaluation and inferences for management of aortopathy patients.

Echocardiographic Z-score models play a crucial role in defining cardiac pathology in paediatric patients. There are multiple models that practitioners utilize in the United States without guiding principles to standardize their use. Discrepant interpretations can occur depending on the model chosen, even if standardized Z-score cutoffs are applied. In this study, a survey was developed to assess pediatric cardiologists' use of Z-score systems when evaluating and treating patients with isolated bicuspid aortic valve. The majority of respondents reported using Z-score cutoffs to evaluate the degree of aortic root and ascending aorta dilation. For the aortic root, mild, moderate, and severe dilation averaged at 2.13 (SD = 0.32), 3.59 (SD = 0.49), and 5.11 (SD = 0.84), respectively. Similar cutoffs were reported for determining ascending aorta dilation. A large proportion of respondents primarily used the Boston system (36%, 18/50) or Boston and Pediatric Heart Network systems together (36%, 18/50). There were also differences in management decisions, such as implementing competitive sports restrictions, based on Z-scores and the 2015 Task Force 7 Bethesda exercise guidelines. These survey results demonstrate variability that exists among paediatric cardiologists in their use of Z-scores for describing aortic root and ascending aorta dilation in patients with isolated bicuspid aortic valve and suggests the need for implementation of national guidelines for Z-score usage.

A rare neurological association of bicuspid aortic valve: A case report

A 45-year-old male presented to the Emergency Department with a 2-h history of sudden onset right-sided headache and difficulty walking. Initial non-contrast computed tomography (CT) of the brain was unremarkable. Twelve-lead electrocardiogram showed left ventricular (LV) hypertrophy with strain pattern. On echocardiography, the aortic valve was noted to be bicuspid, associated with at least moderate eccentric aortic regurgitation and moderate aortic stenosis. In view of the initial presentation of ataxia and headaches, and the finding of bicuspid aortic valve (BAV) on echocardiography, an urgent CT of aorta and carotid arteries was performed, revealing dissection of the right vertebral artery and a right lateral medullary infarct. Aortic root dilatation and aortic dissection are well known complications of BAV. However, vertebral artery dissection in the context of BAV with a normal aortic root is unusual, and to our knowledge has only been reported once in the literature so far.

Ethnic Differences in Ascending Aorta Dimensions and Dilatation Rates: A Systematic Review.

Ethnic differences may substantially influence the morphological characteristics of the ascending aorta, with potential implications for clinical assessment and management of aortic dilatation. This systematic review evaluated the impact of ethnicity on ascending aorta diameter and dilatation rates, highlighting the need for more tailored, ethnicity-specific care in cardiovascular practice. We identified 11 studies that measured ascending aorta dimensions using transthoracic echocardiography (TTE), computed tomography (CT), or magnetic resonance imaging (MRI). Most investigations focused on Asian, Caucasian, African American, and Hispanic populations. Data extraction revealed notable variability in baseline aortic diameters across ethnic groups. Some studies found no significant differences between Asian and Caucasian participants, whereas others reported consistently larger diameters in Chinese ethnicities compared to Caucasians and smaller diameters in African American groups. One investigation, for instance, showed that Chinese participants had ascending aorta diameters approximately 1.5 mm larger than their Caucasian counterparts. Dilatation rates also diverged: one study observed that non-White race was linked to earlier or more rapid aortic root dilation in younger populations. At the same time, another reported that Vietnamese individuals had nearly twice the annual growth rate of ascending aorta dilatation when compared to other ethnicities. Available data on prevalence varied, with some studies suggesting ascending aorta dilatation ranged from about 1.2%to 7.5%in Caucasians, 0.9%to 6.4%in African Americans, and 0.8%to 5.9% in Asians. These findings potentiate the role of ethnicity in shaping aortic dimensions, possibly through a combination of genetic predisposition, environmental factors, and lifestyle influences. Incorporating ethnic background into risk stratification may improve the accuracy of clinical assessments and help guide personalized management strategies for ascending aorta dilatation. Future research should address heterogeneity in measurement techniques, more consistently defining ethnic groups, and explore long-term outcomes to clarify whether these observed morphological differences translate into variations in morbidity and mortality.

Aortic and arterial manifestations and clinical features in TGFB3-related heritable thoracic aortic disease: results from the Montalcino Aortic Consortium

BackgroundPathogenic variants in TGFB3 may lead to a syndromic genetic aortopathy. Heritable thoracic aortic disease (HTAD) and arterial events may occur in TGFB3-related disease but there are limited outcomes data...

The first experience of intraoperative myocardial elastography in cardiac surgery patients

The goal the work was to study the possibility of using elastography on an open heart to determine the stiffness of the left ventricular myocardium. Material and methods. Intraoperative elastography was performed in 6 patients with isolated aortic stenosis and dissecting aneurysm of the ascending aorta with aortic insufficiency. Three patients underwent surgery to replace the aortic valve with mechanical prostheses (SIM-19) and three were operated to replace the ascending aorta with an artificial prosthesis with aortic valve replacement (David’s operation). The average age of the patients was 42±9 years (42–53) years. All patients underwent surgery under conditions of artificial blood circulation. Initially, elastography was evaluated on a working heart, and then on full artificial circulation. The study was performed on a VK 5000 ultrasound device with an intraoperative «stick» type sensor at a frequency of 7.5–15 Mhz, gain of 1.6 Db, resolution of 127 hz. The deformation coefficient was evaluated. The imaging program was exposed as for neurosurgery with a frequency of 15 Mhz. Visualization was performed in B-mode, followed by obtaining shear wave elastography with calculation of the deformation coefficient. Results. Wave elastography was evaluated for various heart pathologies with different myocardial thickness. It was found that the stiffness in the studied areas of the myocardium is different. Thus, in patients with atherosclerotic aortic stenosis and a pressure gradient of more than 100 mmHg, the deformation coefficient was increased, in accordance with the thickness of the myocardium and amounted to 3.81–4.06, and in patients with aortic root dilation and aortic insufficiency, the deformation coefficient was 1.64–2.9. Conclusion. Intraoperative assessment of the left ventricular myocardial deformation coefficient is possible only on a stopped heart and gives an idea of the state of the heart muscle with the possibility of soft and hard areas. Shear wave elastography provides information about the elasticity and hardness of the tissue, which indirectly reflects the viscosity of the myocardium. This study was aimed at verifying the methodology for assessing the characteristics of the elasticity of the left ventricular myocardium for myocardial overload by pressure (aortic stenosis) and volume in case of a dissecting aortic aneurysm with aortic insufficiency.

Surgical Treatment of the Aortic Coarctation in Adults: Extra Anatomical Ascending-to-descending Aortic Bypass and Concomitant Procedures

Objectives: Aortic coarctation is defined as the narrowing of the aortic lumen secondary to hypertrophy of the posterolateral aortic wall’s media layer. The coarctation is locates between the junction from ductus arteriosus to the left subclavian artery and left hemi aortic arch. Aortic coarctation provokes many severe and lethal complications, such as severe aortic stenosis, aortic aneurysms, pseudoaneurysms, stroke, and premature coronary artery disease, unless it had not been repaired in the meantime the initiation of the symptoms. Methods: We presented the consecutive nine patients who underwent aortic coarctation open surgical repair between 2015 and 2020 which initiated ascending-to-descending aortic bypass and then concomitant operations for the secondary cardiac pathology such as ascending aortic aneurysm, Type A aortic dissection and sinus valsalva aneurysm required in some cases. Patients were a median age of 26 years old (range, 19–41 years). About 11% of the patients (n=1) presented to our emergency room with severe chest pain and acute Type 2 aortic dissection was diagnosed by the surgical team, and he underwent operation immediately. The supracoronary aortic replacement and ascending to descending aortic bypass procedures were performed sequentially. About 44% of the patients (n=4) were diagnosed as isolated aortic coarctation and only ascending –to- descending aortic bypass was performed electively. About 22% of the patients (n=2) had ascending aortic aneurysm without aortic valve pathology. These patients underwent supracoronary aortic replacement as concomitant procedure to ascending to descending aortic bypass. About 22% of the patients (n=2) had bicuspid aortic valve and sinus Valsalva aneurysm; therefore, the Bentall procedure was implemented concomitantly. Results: There were not any early deaths reported. Any neurologic complications including neither paraplegia nor stroke revealed during post-operative follow-up. About 88% of patients (n=8) were discharged with stable hemodynamic parameters in 2 weeks. About 11% of these patients (n=1) who underwent Bentall procedure concomitantly had third-degree atrioventricular block as a serious rhythm problem then a permanent pacemaker implanted. This patient had been discharged with stable hemodynamic parameters and pacemaker settings were controlled by the cardiology department. Conclusion: The extra anatomical ascending-to-descending aortic bypass is a safe and feasible method for the open surgical coarctation repair, and it provides concomitant procedures for other cardiac pathologies, which require open surgical treatment.

Intrinsic GATA4 expression sensitizes the aortic root to dilation in a Loeys–Dietz syndrome mouse model

Loeys–Dietz syndrome (LDS) is a connective tissue disorder caused by mutations that decrease transforming growth factor-β signaling. LDS-causing mutations increase the risk of aneurysm throughout the arterial tree, yet the aortic root is a site of heightened susceptibility. Here we investigate the heterogeneity of vascular smooth muscle cells (VSMCs) in the aorta of Tgfbr1M318R/+ LDS mice by single-cell transcriptomics to identify molecular determinants of this vulnerability. Reduced expression of components of the extracellular matrix–receptor apparatus and upregulation of stress and inflammatory pathways were observed in all LDS VSMCs. However, regardless of genotype, a subset of Gata4-expressing VSMCs predominantly located in the aortic root intrinsically displayed a less differentiated, proinflammatory profile. A similar population was also identified among aortic VSMCs in a human single-cell RNA sequencing dataset. Postnatal VSMC-specific Gata4 deletion reduced aortic root dilation in LDS mice, suggesting that this factor sensitizes the aortic root to the effects of impaired transforming growth factor-β signaling.

Long-term outcomes comparison of Bentall-De Bono-versus valve-sparing aortic root replacement: An updated systematic review and reconstructed time-to-event meta-analysis

BackgroundFor patients with aortic root dilatation and a structurally normal aortic valve (AV) undergoing composite aortic valve-graft (Bentall-De Bono) versus valve-sparing aortic root replacement (VSARR) procedures there are conflicting data regarding early and long-term benefits. We undertook a study-level meta-analysis to compare the results of both procedures. MethodsThree databases were assessed, and both randomized trials and observational studies were considered eligible. Kaplan-Meier curves of long-term survival and reoperation risk were reconstructed and compared with Cox linear regression and incidence rate ratios (IRR) with 95 % confidence intervals (CI). Landmark analysis and time-varying hazard ratio (HR) were analyzed. Odds ratios (OR) were calculated for early mortality, postoperative stroke, and re-exploration for postoperative bleeding. A random effects model was used. Sensitivity analyses included leave-one-out-analysis, meta-regression and subgroups analysis. Results1456 articles were identified, including 39 observational studies, totaling 14,651 patients (Bentall-De Bono = 9557 and VSARR = 5094). Twelve studies were adjusted. The mean weighted follow-up was 5.05 ± 3.7 years. VSARR was associated with significantly greater survival (HR = 0.50; 95 % CI, 0.45–0.57; p < 0.0001) at 15-year follow-up. The reoperation risk was higher following VSARR (HR = 1.30; 95 % CI, 1.03–1.63; p = 0.02.), although time-varying HR model and landmark analysis reported an increased risk of reoperation within 5 years after VSARR (HR = 1.57; 95 % CI, 1.23–2.01; p < 0.001), after which the difference disappeared. Subgroups analysis of studies excluding aortic dissection showed a comparable rate of late reoperation. ConclusionsVSARR is associated with improved long-term survival compared to Bentall-De Bono. The risk of late reoperation is higher within 5 years following VSARR, after which the two procedures are comparable.

Abstract 4118882: Decompensated Heart Failure Secondary to a Non-Coronary Sinus of Valsalva to Right Atrium Fistula: A Case Report

Introduction: Sinus of Valsalva aneurysm (SVA) accounts for 3.5% of all congenital cardiac anomalies. Rupture occurs in 35% of cases and can lead to life-threatening fistulas, predominantly involving the right ventricle or right atrium. Description of Case: 62-year-old female with hypertension and hypothyroidism presented to the ER with progressively worsening dyspnea over the past week. She also reported worsening lower extremity edema, orthopnea, and paroxysmal nocturnal dyspnea. Chest X-ray showed large pleural effusions. Her ejection fraction was 66% ejection fraction with a fistula between the right sinus of Valsalva and the right atrium on transthoracic echocardiogram (TTE) which was also seen on transesophageal echocardiogram (TEE). Her heart failure was due to the fistula as she had no coronary artery disease on coronary angiogram. She had cardiothoracic surgery for fistula repair. Postoperatively, she developed complete heart block requiring a permanent pacemaker. She had no further complications and was discharged home in good condition. Discussion: SVA affects the right sinus (94%), with the non-coronary (5%) and left sinus (1%) being less commonly involved. Aneurysm rupture typically forms a fistula into the right ventricle (60%), right atrium (29%), left atrium (6%), or left ventricle (4%), and rarely into the pericardial cavity (1%). Symptoms vary by rupture size and location. TTE is the initial diagnostic tool but has limited sensitivity (50%) for detecting fistulas, whereas TEE has a 90% sensitivity. Cardiac MRI and CT can further refine diagnoses, and cardiac catheterization assesses coronary arteries preoperatively. Surgical closure with a patch is the primary treatment for ruptured SVA, significantly improving survival rates compared to non-surgical management.

Abstract 4148073: Expect the Unexpected: Type A Aortic Dissection in Pregnant Patient with Marfan Syndrome

Case Description: A 26 yo woman with Marfan Syndrome (MFS, FBN1 ), ectopia lentis, aortic dilation and no family history of MFS or aortic dissection was evaluated for preconception counseling. She was asymptomatic, and the size of her aortic root (~4cm) and ascending aorta (~3.5cm) had been stable for over 5 years. She was counseled that risk of cardiac events during her pregnancy is high, but lower compared to those with aortic sizes &gt;4.5cm. Once pregnant, she was followed closely with imaging every trimester (MRA at 25 weeks gestation showed stable aortic sizes). Cardio-obstetric recommendations included: CARPREG II score: 2, mWHO class: III, consideration of assisted second stage due to aortic root dilation. She was counseled on symptoms of aortic dissection and to seek emergency care should those symptoms arise. At 27 weeks gestation, she presented to the ED with intense chest and neck pain, shortness of breath, and headache. Emergent CT showed aortic root (4.1cm) and proximal ascending aortic dissection with propagation into the left main coronary artery (Figure 1). She underwent emergent C-section followed by aortic root and ascending aorta replacement and mechanical aortic valve replacement due to severe regurgitation. She was discharged in stable condition 8 days after an uncomplicated post-operative recovery. Her baby is recovering in the NICU. Discussion: While risk of aortic dissection in women with MFS is higher during pregnancy, recent studies suggest relative safety with aortic root diameters up to 4.5cm. Our case highlights that even at “lower risk” aortic sizes, aortic dissection remains an important risk for such women. Educating patients and providers about the acute symptoms of aortic dissection and forming an action plan for timely intervention can be lifesaving. Furthermore, some genetic mutations (e.g. TGBR1/2 or SMAD3 ) and sequence variants within the FBN1 gene may pose a higher risk of aortic dissection at smaller aortic diameters. Our patient’s pathogenic FBN1 mutation (-c.2114-5T&gt;G, previously classified as a VUS) highlights the importance of genetic testing: prophylactic surgery at sizes &lt;4.5cm may be considered with high-risk genetic variants or a family history of aortic dissection.

Echocardiographic Assessment in Patients with Vascular Ehlers-Danlos Syndrome: Insights from an Unexplored Field.

Vascular Ehlers-Danlos syndrome (vEDS) is an inherited connective tissue disorder characterized by arterial fragility. Celiprolol is a β1-adrenoceptor antagonist with partial β2 agonist activity that has been shown to reduce rates of vascular events in this setting, though the underlying mechanisms are not yet fully understood. Moreover, very few echocardiographic data are available in patients with vEDS. To perform a comprehensive echocardiographic assessment of a cohort of patients with vEDS with or without celiprolol therapy compared with healthy subjects. Twenty patients with genetically confirmed diagnosis of vEDS followed at our Institution (University Hospital of Brescia, Italy) were divided into two groups according to whether or not they were on celiprolol therapy at the maximum recommended dose (400mg daily) for at least 12 months. Both groups were compared to 10 healthy individuals matched for sex, age, body mass index (BMI), and office blood pressure (BP) values. Each participant underwent transthoracic echocardiography with tissue Doppler analysis (TDI) for a comprehensive evaluation of cardiac structure and function. Mean age was 35 years and mean BMI was 21.6 kg/m2. Female sex was prevalent (60%). Left ventricular (LV) internal diameter values tended to be lower in patients with untreated vEDS than in healthy controls (4.33 vs 4.74cm, respectively), though this difference was not statistically significant. Similar data were observed for LV mass index (56.9 vs 68.6 g/m2), stroke volume (56.6 vs 71.6 mL), and E/A ratio (1.26 vs 1.66), whereas an opposite trend was observed for e' lateral (13.2 vs 12.2cm/s). No statistically significant difference was found between groups in terms of other parameters of LV mass, systolic and diastolic function. A normal LV geometry was found in all the cases. Indices of mechano-energetic efficiency and ventricular-arterial coupling were also similar between groups. No patient presented with aortic root dilation, mitral valve prolapse, valve insufficiency of more than mild degree, or valve stenosis of any degree. Our study suggests that patients with vEDS have normal cardiac mass and geometry, as well as normal systolic and diastolic function. Celiprolol therapy does not seem to significantly influence such aspects. Compared with vascular imaging, less stringent follow-up with echocardiography seems reasonable in this setting. Future studies with prospective design should confirm these aspects.

2D Echocardiographic aortic root dimensions and longitudinal analysis with artificial intelligence using an open dataset from a UK-wide collaborative

Abstract Background Surveillance of aortic dimensions requires reproducible measurements, and knowledge of the rate of progression in those with dilatation. Purpose To develop an open machine-learning method for measuring the aortic root and proximal ascending aorta on echocardiograms, validate it through a multi-expert panel from the Unity UK Echocardiography AI Collaborative, and applying the AI to derive the rate of progression in historical databases. Methods The neural network was trained on 1478 parasternal long-axis images. Each image was labelled with key points for the aortic annulus, sinus and sinotubular junction, and proximal ascending aorta dimensions. Labels accommodated inner-to-inner and leading-to-leading conventions. For validation, the end-diastolic and mid-systolic images were identified (accommodating different guidelines recommendations) from 100 studies, and 10 expert echocardiographers made the aortic measurements. The consensus of experts defined the reference standard, and the variation between individual experts defined the acceptable variation for the AI. The AI was then applied to 724 echocardiograms of 102 patients under surveillance spanning 12 years (ranging from 4 to 17 scans per patient) without intervention. Training data labels and networks are made freely available on our project website. Results The median absolute deviations between the AI and the expert consensus ranged from 0.06cm to 0.17cm across the 16 measurements. For the individual expert opinions this was from 0.08cm to 0.19cm. For 7/16 measurements, there was no statistically significant difference between the AI deviation and the individual-expert deviation. The AI’s confidence level was a useful indicator of the reliability of the AI measure. For the top 9 deciles of AI confidence level, the AI measurements had significantly smaller deviations than the individual experts. The rates of progression averaged across the three aortic measurements was 2.0 mm/decade (95% CI 1.0 to 3.0; p =0.007). Conclusion Expert consensus can be used to both define the reference standard and also the range of acceptable deviation from consensus. Overall, the AI performs similarly to experts and more importantly, provides an automated estimate of confidence. The validated AI is consistent over time, and when applied retrospectively to patients with dilated aortic roots, found the rate of progression was more rapid than in previously reported healthy patients. This approach could be applied generally in developing medical AI to not only make reproducible measurements but to automate large scale longitudinal studies with little input, and ultimately serves as a useful research and clinical tool.Figure: AI(red) vs Expert (blue)Table: AIDeviations from Consensus

Distal ascending aortic dilatation portends a near 2-fold greater risk of cardiovascular mortality than proximal enlargement: results from 120,000 patients in a nationwide echocardiographic database

Abstract Background The management of thoracic aortic dilatation is clinically challenging and acute aortic dissection (AD) is under-recognised as a primary cause of death. Despite the majority of AD occurring in non-severely dilated aortas, absolute aortic diameter remains the most utilised criterion for adjudging preventative surgical correction. In order to improve outcomes and guide management, greater prognostic understanding is needed. Purpose We examined a nationwide echocardiographic dataset linked to mortality outcomes to determine how ascending thoracic aortic dilatation and its site affects cardiovascular disease (CVD) mortality over time. Methods The National Echocardiography Database Australia (NEDA) was examined for aortic dimensions at the sinuses of Valsalva (SoV), sinotubular junction (STJ) and ascending aorta (AscAo) in patients who had undertaken &amp;gt;1 echocardiographic study. Following separation of those who had undergone corrective aortic surgery, patients were stratified according to absolute aortic diameters and grouped as normal (&amp;lt;4cm), mild (4.0-4.5cm), moderate (4.5-5cm) and severely (&amp;gt;5cm) dilated at the prescribed thoracic aortic sites. Mortality data was then linked from the National Death Index (NDI). Results 398,905 echocardiographs from 121,115 patients with a mean age of 62.6±16.2 years were included. At the time of mortality data linkage, 38,681 (32%) had died, of whom 13,292 (34%) died from CVD causes and only 276 (0.007%) had aortic dissection recorded as their primary cause of death. Severe aortic dilatation at any site increased the likelihood of 10-year CVD mortality (29% vs. 15% in those with normal diameters; HR 3.00; CI 1.60-5.63; p&amp;lt;0.0001), with an incremental increase in the probability of CVD death when moving from the proximal to distal ascending aorta; 10-year CVD mortality at the SoV 26% (HR 1.93; CI 1.29-2.87; p&amp;lt;0.001), STJ 39% (HR 2.82; CI 1.64-4.86; p&amp;lt;0.001) and AscAo 44% (HR 4.74; CI 2.46-9.13; p&amp;lt;0.001) respectively (Figure 1). When examining those patients who had undergone corrective aortic surgery, the likelihood of 10-year CVD mortality was comparable to the population with normal aortic diameters (17 vs. 15%, HR 1.1; CI 0.9-1.36; p=0.3) (Figure 2). Conclusion Severe aortic dilatation increases the probability of 10-year cardiovascular mortality. Interestingly, there is an incremental increase in the likelihood of CVD mortality when moving from the proximal to distal ascending aorta, with severe AscAo dilatation conferring a near 2-fold greater risk of CVD mortality compared with severe SoV aneurysms. Additionally, surgical aortic intervention nearly normalises survival probability to the population with healthy aortic diameters. These results identify new considerations for prognostication, risk stratification and surgical guidance of aortic repair.CVD mortality by aortic dilatationCVD mortality after aortic intervention

Gradual hypertensive response in aortic alterations and myocardial inflammatory activation in progressing HFpEF in mice

Abstract Introduction Hypertension is the most common comorbidity in patients affected by heart failure with preserved ejection fraction (HFpEF). Early effects of pressure overload and its contribution to the gradual development of cardiac and vascular pathology leading to HFpEF are not well recognized, as patients are diagnosed when the disease has already advanced and show symptoms. Several hypertensive HFpEF mouse models possessing the hallmarks of the clinical-like phenotype are available. Nonetheless, initial and gradual pathological manifestations of hypertensive HFpEF have not been comprehensively described. Purpose This study aimed to investigate the gradual progression of HFpEF phenotype development in mice from the early manifestation of cardiac and vascular pathology to global cardiac dysfunction to multiorgan syndrome in response to hypertension. Methods Hypertensive HFpEF was induced by angiotensin II infusion in C57BL/6JOlaHsd 18-20-week-old male mice that were given low, moderate and high doses (0.5mg/kg/day - 1.5 mg/kg/day) subcutaneously for 4 weeks. Cardiac physiology and alterations in haemodynamics were assessed with echocardiography of heart and aorta, weekly ECG monitoring, and blood pressure measurements from the mouse tail. Extensive histology and immunohistochemistry analyses of cardiac tissue, microvasculature, lungs and kidneys were performed. Results In the low dose group, loss of aortic wall flexibility and presence of aortic root dilatation were detected. This group also demonstrated initiation of concentric remodelling process with the upsurge in macrophage infiltration in myocardium, but the signs of diastolic dysfunction were not yet present. The high dose group exhibited characteristic HFpEF features with diastolic dysfunction, left ventricular hypertrophy and fibrosis. Pathology in cardiac conduction system was noticed in all groups, with prominent ECG waveform abnormalities already on week one. However, only the high dose group experienced non-recovery in impulse propagation, accompanied by significant prolongation of ventricular depolarization. Conclusion This study demonstrates that change in aortic root dimensions and aortic wall dynamics are the first hypertension-sensitive alterations together with myocardial inflammatory cell infiltration that may contribute to myocardial remodelling and subsequent development of HFpEF and systemic effects. Hypertensive angiotensin II-induced HFpEF is a relevant mouse model, recapitulating not only hallmarks of cardiac pathology but also an extensive range of vascular and multiorgan alterations, demonstrating similarities with the clinical phenotype.