Sinus Of Valsalva Aneurysm Research Articles

Valsalva Aneurysm of Right Sinus: A Case Report and Review of Literature

In this case report, we describe the diagnostic modality of sinus of Valsalva aneurysm (SOVA) in combination with congenital cardiac defect, aortic valve involvement, and conduction abnormality in a 19-year-old patient. Aim of article was to understand the importance of clinicians being cautious about SOVA presenting in young patients, despite cases being rare, and that SOVA requires a thorough SOVA diagnostic approach. We further provide a review of literature highlighting and comparing the treatment options for both unruptured and ruptured SOVAs. The patient presented for examination due to tachycardia and palpitations. A murmur was heard, and the patient was found to have an atrioventricular nodal reentry tachycardia. Echocardiographic evaluation, magnetic resonance imaging, and computed tomography angiography confirmed an aneurysmally dilated aortic root, aortic regurgitation, and ventricular septal defect. Surgical intervention was indicated; however, the patient refused to undergo surgery.

Aortic valve cusp size and shape in dilated trileaflet aortic roots

ObjectivesThe objective of the study was to quantify the differences in cusp size and shape in patients with normal and dilated trileaflet aortic roots and in dilated roots with or without aortic regurgitation. MethodsA retrospective analysis of computed tomography studies in patients with normal and dilated trileaflet aortic roots was performed measuring root and cusp dimensions. Normal root size was defined as sinuses of Valsalva diameter less than 40 mm, dilated as 45 mm or greater. Root measurements normalized to basal ring diameter and cusp measurements normalized to geometric height were analyzed to assess the shape. Additionally, comparison of dilated roots with or without aortic regurgitation was made. ResultsWe analyzed 146 normal and 104 dilated aortic roots and 73 propensity-matched pairs. Dilated roots were larger in all dimensions and had increased ratio between commissural and basal ring diameter (1.58 ± 0.23 vs 1.11 ± 0.10, P < .001). Cusps in dilated roots were larger in all measured dimensions and were elongated with increased normalized cusp insertion length (3.64 ± 0.39 vs 3.26 ± 0.20, P < .001) and normalized free margin length (2.53 ± 0.30 vs 2.16 ± 0.19, P < .001). In patients with dilated root and no cusp prolapse (n = 83), those with moderate or severe aortic regurgitation had larger commissural diameter but similar cusp dimensions compared with those with no or mild aortic regurgitation. ConclusionsThe cusps in dilated roots elongate transversely and to a lesser degree radially. Functional aortic regurgitation is caused by extensive commissural dilatation and not by inadequate cusp adaptation.

Reoperative aortic root surgery: single-center long-term outcomes and literature review.

Reoperative aortic root surgery has become more and more common over the years and is considered high-risk, with significantly worse outcomes compared to first-procedure root surgery. At our institution, this kind of surgery is frequently performed. The aim of the present review is to describe currently available literature on reoperative surgery on the aortic root in terms of patients' population, indications for surgery and outcomes and to present our center's experience on the matter. A literature review was performed in order to identify pertinent studies. They were then compared and described. We also described preoperative characteristics, operative strategies and outcomes of all the patients who underwent redo aortic root surgery from January 1986 to December 2022 at our center. Our literature review identified 12 pertinent studies, with a total of 16,627 considered patients. The most frequent indications for redo surgery were endocarditis (35.5%), aneurysm, dissection and pseudoaneurysm. Mean cardiopulmonary bypass (CPB) and cross-clamp times were 218 and 152 minutes, respectively. In-hospital mortality was 12%. When analyzing our center's data, 344 procedures were identified. Aortic root dilation was the most frequent indication (36.9%). Mean CPB and cross-clamp times were 218.0±78.8 and 158.2±49.7 minutes, respectively. In-hospital mortality was 9.6%. Survival at 5 and 15 years was 76.1% and 51.4% respectively. Freedom from further aortic reintervention was 88.1% after 5 years and 64.9% after 15 years. Reoperative aortic root surgery is a difficult cardiac procedure which is linked to significantly higher mortality than first-time root replacement. If it is performed by experienced surgeons with a careful preoperative planning its result can still be satisfactory. Our results showed acceptable rates of mortality and reinterventions at follow-up. Endocarditis, however, was linked to worse outcomes.

Intrinsic Gata4 expression sensitizes the aortic root to dilation in a Loeys-Dietz syndrome mouse model.

Loeys-Dietz syndrome (LDS) is an aneurysm disorder caused by mutations that decrease transforming growth factor-β (TGF-β) signaling. Although aneurysms develop throughout the arterial tree, the aortic root is a site of heightened risk. To identify molecular determinants of this vulnerability, we investigated the heterogeneity of vascular smooth muscle cells (VSMCs) in the aorta of Tgfbr1 M318R/+ LDS mice by single cell and spatial transcriptomics. 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 scRNAseq 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 TGF-β signaling.

#1607 Prevalence and risk factors of aortic root dilatation in children with X-linked Alport syndrome

Abstract Background and Aims Aortic dilatation in X-linked Alport syndrome (XLAS) is considered a specific manifestation. On the other hand, all patients with CKD have been shown to have an increased risk of aortopathies. The aim was to define prevalence and risk factors for aortic dilatation in children with XLAS. Method Retrospective cross-section single center study included XLAS’ children (n = 98,64 M) and patients with CACUT (comparison group, n = 26,12 M). The aorta was measured at level of the sinus of Valsalva (SoV), aortic dilatation was determined by z-score &amp;gt;2 for BSA. Body mass index (BMI, kg/m2), z-scores of mean systolic blood pressure (SBP), mean diastolic blood pressure (DBP), mean blood pressure (MBP), proteinuria (Pr, mg/m2/day), eGFR (ml/min/1.73 m2), z-scores of left ventricular mass index (LVMI), left ventricular end diastolic diameter (LVEDD), relative wall thickness of the left ventricle (RWT, N &amp;lt; 0,42) data were obtained. Results There were no difference in age (11,12 ± 3,75 vs 10,87 ± 2,34 yrs; p = 0,78), gender (males 0,55 vs 0,46; p = 0,45), BMI (18,7 ± 3,7 vs 19,1 ± 4,7; p = 0,78), eGFR (103,1 ± 16,9 vs 90,5 ± 11,4; p = 0,50), prevalence of hypertension (0,11 vs 0,19; p = 0,21) between the groups. The prevalence of SoV dilatation did not differ between XLAS and CACUT (0,06 vs 0,08; p&amp;gt;0,18). SoV dilatation was associated with male gender (β1 = 0,31, p = 0,02; β2 = 0,34, p = 0,02), LVMI (β1 = 0,26, p = 0,02; β2 = 0,24, p = 0,02) in both groups, with BMI (β1 = -0,27, p = 0,03), LVEDD (β1 = 0,21, p = 0,04) in children with XLAS. No statistically significant effect of SBP, DBP, MBP, Pr, eGFR on SoV dilatation has been demonstrated. Conclusion The prevalence of SoV dilatation in children with XLAS was higher than in general population, but comparable to children with CACUT. The male gender, low BMI, LVMI and LVEDD were associated with SoV dilatation in XLAS. We did not show the relationship between blood pressure load, proteinuria, eGFR and SoV dilatation in XLAS.

Transcatheter Aortic Valve Implantation for Severe Chronic Aortic Regurgitation.

Transcatheter aortic valve implantation (TAVI) has revolutionised the management of aortic valve disease, offering a less invasive alternative to traditional surgical valve replacement for severe aortic stenosis (AS). TAVI for pure aortic regurgitation (AR) is less well established, and, in fact, it was previously labelled as a relative contraindication. However, TAVI has been utilised for selected cases of pure or predominant AR. The primary limitations regarding the use of TAVI in AR are related to the absence of anatomical factors seen in patients with AS that have contributed to the safe and stable functioning of current-generation prostheses. These include aortic root dilatation, mobile valve leaflets and labile blood pressure within the aortic root, which may further increase the risk of valve migration and periprosthetic leak after deployment. Furthermore, patients with AR have more heterogeneous aortic root anatomies when compared to the population of patients with calcific or degenerative AS. This review article describes the current evidence for the off-label use of TAVI in pure AR and the various clinical syndromes associated with AR where there may be specific challenges in the application of TAVI.

Mitral annular disjunction and its progression during childhood in Marfan syndrome.

Data on mitral annular disjunction (MAD) in children with Marfan syndrome (MFS) are sparse. To investigate the diagnostic yield of MAD by echocardiography and cardiac magnetic resonance imaging (CMR), its prevalence and progression during childhood. We included patients <21 years old with MFS, defined by 2010 Ghent criteria and a pathogenic FBN1 variant or ectopia lentis. Two readers measured systolic separation between the mitral valve (MV) posterior hinge point and left ventricular (LV) myocardium on initial and subsequent imaging. MAD was defined as MV-LV separation ≥2 mm, MV prolapse (MVP) as atrial displacement ≥2 mm. Kappa coefficients evaluated echocardiogram-CMR agreement. Bland-Altman and intraclass correlation coefficients (ICCs) assessed inter-rater and inter-modality reliability. Univariable mixed-effects linear regression was used to evaluate longitudinal changes of MAD. MAD was detected in 60% (110/185) eligible patients. MVP was present in 48% (53/110) of MAD and MAD in 90% (53/59) of MVP. MAD detection by CMR and echocardiography had 96% overall agreement (Kappa = 0.89, P < 0.001) and a 0.32 mm estimate bias (95% CI 0.00, 0.65). ICC by echocardiography, CMR, and between modalities were 0.97 (95% CI 0.93, 0.98), 0.92 (95% CI 0.79, 0.97), and 0.91 (95% CI 0.85, 0.94), respectively. MAD was associated with aortic root dilation (P < 0.001). MAD was found in children of all ages, increased +0.18 mm/year (95% CI +0.14, +0.22) during a median duration of 5.5 years (IQR 3.1, 7.5 years). MAD indexed by height yielded a constant value +0.0002 mm/m/year (95% CI -0.0002, +0.0005 mm/m/year). MAD was common in pediatric MFS and was associated with aortic root dilation. MAD detection by echocardiography and CMR was highly reliable, suggesting that routine assessment in MFS is feasible. MAD was present in neonates and progressed over time but remained constant when indexing by height. Further studies are needed to evaluate MAD as a biomarker for clinical outcomes in pediatric MFS.

Rupture of sinus of Valsalva aneurysm in a young adult male: A case report

Rupture of the sinus of Valsalva aneurysm (SVA) is a surgical emergency. It can be fatal if not treated properly and on time. In this report, we present a 39-year-old male patient with no known disease who was admitted to the emergency department with complaints of pressure-such as chest pain, chest tightness, and an inability to breathe easily after a heavy meal at night. Thoracic and abdominal computed tomography angiography was performed to better evaluate the vascular structures; the ascending aorta diameter was 71 mm (wider than normal). Hemorrhagic fluid was observed in the pericardial space and paracardiac recesses. The preliminary diagnosis was a rupture of the right SVA, and emergency surgery was performed. The posterior aorta was found to have ruptured at the level of the right sinus of Valsalva, and anastomosis was performed on the torn portion. The patient was extubated 8 h after surgery. On the 7th post-operative day, transthoracic echocardiography was within normal limits, and he was discharged from the hospital on the 8th day in good condition with stable vital signs.

FIRST COMPREHENSIVE ECHOCARDIOGRAPHIC ASSESSMENT IN PATIENTS WITH VASCULAR EHLERS-DANLOS SYNDROME WITH OR WITHOUT CELIPROLOL THERAPY AT THE MAXIMUM RECOMMENDED DOSE

Objective: Vascular Ehlers-Danlos syndrome (vEDS) is an inherited connective tissue disorder characterized by arterial fragility. Celiprolol is a beta1-adrenoceptor antagonist with partial beta2 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, few data on echocardiographic assessment are available in patients with vEDS. Design and method: Twenty patients with genetically confirmed diagnosis of vEDS followed at our Institution were divided into two groups (10 patients each) according to whether or not they were on celiprolol therapy at the maximum recommended dose (400 mg 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 values. Each participant underwent transthoracic echocardiography with tissue Doppler analysis for a comprehensive evaluation of cardiac structure and function. Results: 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, though this difference was not statistically significant (4.33 vs 4.74 cm, respectively; p=0.053). Similar data were observed for LV mass index (56.9 vs 68.6 g/m2; p=0.093), stroke volume (56.6 vs 71.6 mL; p=0.068), and E/A ratio (1.26 vs 1.66; p=0.082), whereas an opposite trend was observed for e’ lateral (13.2 vs 12.2; p=0.096). 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. Conclusions: 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. Future studies on larger cohort of patients should confirm these aspects.

Abstract 2009: Computational Modeling Of Long-term Aortic Root Dilatation Following TEVAR Of Type B Aortic Dissection

Objectives: The long-term sequalae of thoracic endovascular aortic repair (TEVAR) are poorly understood. While several failure modes have been identified, the hemodynamic impact of TEVAR in the proximal native ascending aorta has yet to be fully appreciated. This study applies computational modeling tools to investigate a case of long-term aortic root dilatation following TEVAR for acute type B aortic dissection (TBAD). Methods: We investigate a patient who presented with heart failure 4.5 years after initially successful TEVAR. Cross-sectional aortic root diameter was measured in a pre-operative scan and 57 months post-TEVAR. Three-dimensional localized surface dilatation was calculated using pre-operative and post-operative segmentations, followed by a non-rigid surface mapping method of calculating area change in corresponding regions. The hemodynamic impact of TEVAR on the proximal aorta was analyzed with a computational fluid dynamics (CFD) simulation of the post-operative patient geometry. Results: Aortic root diameter increased from 39.2 mm pre-op to 47.1 mm (+ 20.1%) postop and 34.9 mm preop to 40.8 mm (+ 16.9%) postop on axial and coronal images respectively. The three-dimensional area mapping between pre and post-op scans demonstrates a maximum 14% area increase near the aortic root. The CFD simulations show increased velocity and static pressure in the ascending aorta near the aortic root region. Conclusions: Three different computational approaches demonstrate long-term changes in ascending aortic morphology following TEVAR. Computed tomography measurements demonstrate significant aortic root dilatation, and three-dimensional surface mapping shows increasing area in the proximal ascending aorta. The CFD results emphasize localization of increased velocity and stress in the same region. These observations point to potential avenues for further mechanistic investigation.

Diagnostic Accuracy of Transthoracic Echocardiography for Acute Type A Aortic Syndrome: A Systematic Review and Meta-Analysis

Transthoracic echocardiography (TTE) is currently recognized as the potential first-line imaging test for patients with suspected acute type A aortic syndrome (AAAS). Direct TTE sign for detecting AAAS is positive if there is an intimal flap separating two aortic lumens or aortic wall thickening seen in the ascending aorta. Indirect TTE sign indicates high-risk features of AAAS, such as aortic root dilatation, pericardial effusion, and aortic regurgitation. Our aim is to summarize the existing clinical evidence regarding the diagnostic accuracy of TTE and to evaluate its potential role in the management of patients with suspected AAAS. We included prospective or retrospective diagnostic cohort studies, written in any language, that specifically focused on using TTE to diagnose AAAS from databases such as PubMed, EMBASE, MEDLINE, and the Cochrane Library. The pooled sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), diagnostic odds ratio [1], and hierarchical summary receiver-operating characteristic (HSROC) curve were calculated for TTE in diagnosing AAAS. We applied Quality Assessment of Diagnostic Accuracy (QUADAS-2) tool and Grading of Recommendations, Assessment, Development and Evaluation (GRADE) quality assessment criteria. Ten studies (2886 patients) were included in the meta-analysis. The pooled sensitivity and specificity of direct TTE signs were 58% (95% CI, 38-76%) and 94% (95% CI, 89-97%). For any TTE signs, the pooled sensitivity and specificity were 91% (95% CI, 85-94%) and 74% (95% CI, 61-84%). The diagnostic accuracy of direct TTE signs was significantly higher than that of any TTE signs, as measured by the area under the HSROC curve [0.95 (95% CI, 0.92-0.96) vs. 0.87 (95% CI, 0.84-0.90)] in four studies. Our study suggests that TTE could serve as the initial imaging test for patients with suspected AAAS. Given its high specificity, the presence of direct TTE signs may indicate AAAS, whereas the absence of any TTE signs, combined with low clinical suspicion, could suggest a lower likelihood of AAAS.

Case Report: Marfan’s Syndrome with Neurofibromatosis Type-2

Background Marfan’s syndrome (MFS) and neurofibromatosis type-2 (NF-2) are rare autosomal dominant diseases caused due to mutations in chromosome number 15 and 22, respectively. The chance of both the diseases occurring in the same patient is extremely rare. Case presentation In this report a rare case of a 16-year-old boy having a coexistence of both MFS and NF-2 is reported. The clinical findings consisted of musculoskeletal abnormalities consisting of laxity of joints, positive Steinberg thumb sign test, positive Walker-Murdoch wrist test, dolichostenomelia, and arachnodactyly. Anthropometric abnormalities reported an increase in lower segment height than the upper segment. Diagnostic assessment revealed pectus carinatum, multiple meningioma, and heterogeneous lesions in bilateral cerebellopontine angle that were ruled out as bilateral schwannoma confirming the diagnosis of MFS and NF-2. However, both must be regarded as novel mutations, and as the position of the two genes is unrelated, it is most probable that two different mutations have occurred. β-blocker was recommended as a prophylactic measure to minimize aortic root stiffness and control aortic root dilatation. Furthermore, surgery and radiation therapy were planned for the management of NF-2; however, the patient expressed skepticism regarding the course of treatment and follow-ups; as a result, this data was reported at the time of discharge with non-specified duration of follow-ups. Conclusions This case report demonstrates a rare case of MFS which is caused due to mutation in chromosome number 15 along with NF-2 which is caused due to mutation in chromosome number 22. Since the positions of the two genes are unrelated, it is concluded that two distinct mutations have occurred. As a result, this report highlights a rare coexistence of both mutations, which will benefit the medical profession in enhancing future studies.

Long-term outcomes after the arterial switch operation.

The aims of this study were to evaluate the 16-year experience with arterial switch operation at Beijing Children's Hospital and to determine early and late mortality and late morbidity, to explore risk factors for late complications and reintervention, and finally to evaluate whether the neoaorticsinotubular junction reconstruction technique reduces late complications of arterial switch operation. The clinical data of 185 patients with transposition of the great arteries who underwent arterial switch operation in Beijing Children's Hospital from January 2006 to January 2022 and 30 patients who underwent modified arterial switch operation with neoaorticsinotubular junction reconstruction technique in Fuwai Hospital during the same period were retrospectively analysed. Propensity score matching was also used to match the neoaorticsinotubular junction reconstruction patients in Fuwai Hospital with 30 non-neoaorticsinotubular junction reconstruction patients in Beijing Children's Hospital. There were 13 early deaths (7.03%) and five late deaths (3.01%). Nineteen patients (11.45%) developed new aortic valve regurgitation and 28 patients (16.87%) developed aortic root dilation. Late right ventricular outflow tract obstruction occurred in 33 patients (19.88%). Late reintervention occurred in 18 cases (10.84%). Multivariate analysis showed that aorto-pulmonary diameter mismatch, previous pulmonary artery banding, and mild moderate or above new aortic valve regurgitation at discharge were independent risk factors for late new aortic valve regurgitation and aortic root dilation. Low surgical weight was an independent risk factor specific to new aortic valve regurgitation, and bicuspid native pulmonary valve was an independent risk factor specific to aortic root dilation. Older surgical age and aortic root dilation were independent risk factors for late right ventricular outflow tract obstruction. Older surgical age, operation before 2014, late right ventricular outflow tract obstruction, and late aortic root dilation were independent risk factors for late intervention. Propensity score matching showed that new aortic valve regurgitation and aortic root dilation were not followed up in the neoaorticsinotubular junction reconstruction group, while seven cases of aortic root dilation and five cases of new aortic valve regurgitation occurred in the non-neoaorticsinotubular junction reconstruction group, respectively, and the differences were statistically significant (P = 0.003; P = 0.015). The increased incidence of new aortic valve regurgitation, aortic root dilation, and right ventricular outflow tract obstruction as children age is a major concern outcome in the future and may mean more late reintervention. neoaorticsinotubular junction reconstruction technique may reduce the incidence of new aortic valve regurgitation and aortic root dilation, and improve the late prognosis of arterial switch operation. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with aorto-pulmonary diameter mismatch, previous pulmonary artery banding, mild new aortic valve regurgitation at discharge, low surgical weight, and bicuspid native pulmonary valve structures.

Marfan and Loeys-Dietz aortic phenotype: A potential tool for diagnosis and management

ObjectiveIn heritable aortic diseases, different vascular involvement may occur with potential variable implications in aortic dilation/dissection risk. This study aimed to analyze the aortic anatomy of individuals with Marfan syndrome and Loeys-Dietz syndrome to identify possible morphological differences. MethodsComputed tomography and magnetic resonance imaging of the thoracoabdominal aorta from the proximal supra-aortic vessels to the femoral bifurcation level of 114 patients with Marfan and Loeys-Dietz syndromes and 20 matched control subjects were examined. Aortic diameters, areas, length, and tortuosity were measured in different aortic segments using specific vessel analysis software. ResultsPatients with Marfan syndrome showed a higher prevalence of ascending aorta and aortic root dilation (P = .011), larger and longer aortic roots (P = .013) with pear-shaped phenotype, larger isthmus/descending aorta diameter ratio (P = .015), and larger suprarenal aorta and iliac arteries. Patients with Loeys-Dietz syndrome showed longer indexed segments and a significantly longer arch (P = .006) with type 2/3 arch prevalence (P = .097). Measurement ratios analysis provided cut-off values (aortic root to ascending aorta length/aortic root diameter, aortic root/sinotubular junction, aortic root/ascending aorta diameter) differentiating patients with Marfan syndrome from patients with Loeys-Dietz syndrome, even in the early stage of the disease. ConclusionsBoth syndromes show peculiar anatomic patterns at different aortic levels irrespective of aortic dilation and disease severity. These features may represent the expression of different genetic mutations on aortic development, with a potential impact on prognosis and possibly contributing to better management of the diseases. The systematic adoption of whole body imaging with magnetic resonance or computed tomography should always be considered, because they allow a complete vascular assessment with practical indicators of differential diagnosis.

Aortopathy and aortic valve surgery in patients with bicuspid aortic valve with and without raphe

AimTo evaluate the association between raphe in bicuspid aortic valve (BAV) patients and valve dysfunction, aortopathy and aortic valve surgery in the REBECCA registry [REgistro della valvola aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging (SIECVI)]. MethodsPrevalence of aortic valve dysfunction and aortopathy was investigated in BAV patients with and without raphe. Aortic valve dysfunction (regurgitation or stenosis) was categorized as mild, moderate and severe. Aortopathy was defined as annulus ≥14 mm/m2; root ≥20 mm/m2; sinotubular junction ≥16 mm/m2; ascending aorta ≥17 mm/m2, and classified in Type A, isolated ascending aorta dilatation; Type B, aortic root and ascending aorta dilatation; and Type C, isolated aortic root dilatation. ResultsOverall, 695 patients with BAV were enrolled; 520 (74.8%) with raphe and 175 (25.2%) without raphe. BAV patients with raphe presented more frequently with moderate or severe aortic stenosis than BAV patients without raphe (183 [35.2%] vs 34 [19.4%], p < 0.001). A higher prevalence of aortopathy, particularly Type B, was observed in patients with vs without raphe. At multivariable analysis, raphe was a predictor of aortic valve surgery at three-year follow-up (odds ratio 2.19, 95% confidence interval 1.08–4.44, p < 0.001). ConclusionsPatients with BAV and raphe have a higher prevalence of significant aortic stenosis, aortopathy, especially Type B, and a higher risk of undergoing aortic valve surgery at three-year follow-up.

Surgical preference for aortic root and ascending aortic aneurysm in pediatric patients: single-center experience

Purpose: Aortic root and ascending aortic aneurysms are rarely encountered in pediatric patients. Our aim in this study was to compare our surgical approach and results in this patient group with similar studies in the literature.&#x0D; Materials and Methods: This study retrospective in design and its cohort consists of pediatric patients who were operated on due to the diagnosis of aortic root and/or ascending aortic aneurysm. The etiologies of the patients that caused the aneurysm were investigated preoperatively, and the severity of the disease in the aortic valve and ascending aorta and surgical indications were revealed with detailed imaging methods. Operative data was then retrieved and early postoperative morbidity and mortality, as well as post-discharge aortic valve functions were evaluated. The obtained data was compared with those of similar articles in the literature.&#x0D; Results: A total of eight pediatric patients were operated on with the diagnosis of aortic root and ascending aortic aneurysm. The average age of the patients was eleven (±4.03) years. The underlying cause of three (37.5%) patients was Marfan syndrome, two (25%) had bicuspid aortic valve and one (12.5%) had Wiscott-Aldrich Syndrome, previously operated sinus valsalva aneurysm and operated truncus arteriosus. There was 3rd degree aortic valve insufficiency in six patients (75%). The average aortic annulus diameter, mean sinus of valsalva diameter and ascending aorta diameter of the patients were 26.7 mm (±5.3), 40.9 mm (±9.7) and 37.8 mm (±9.2), respectively. The David I procedure was performed in three (37.5%) patients, while the Bentall procedure or aortic valve and ascending aorta replacement was performed on the other five patients. Non-cardiac mortality was observed in one (12.5%) patient, whereas native and mechanical valve dysfunction was not observed in any patient after discharge.&#x0D; Conclusion: Both valve-sparing surgical procedures and other replacement techniques can be used safely in pediatric patients with aortic root and ascending aortic aneurysms.

Sinus of Valsalva Aneurysm: Different Modes of Presentation and Techniques of Repair.

A sinus of valsalva aneurysm (SVA) is an asymmetrical dilatation of the aortic root located between the aortic valve anulus and the sino-tubular junction. Congenital weakness of the elastic lamina in the aortic media layer or trauma and infection as acquired events are the most principal causes of SVA. Presentation may be acute when rupture has occurred or SVA may be discovered fortuitously on echocardiography or CT scan when patients are examined because of unspecific chest pains, dyspnea or arrhythmias. Although endovascular treatment has been performed successfully in individual cases, surgical closure of the aneurysm aiming at preservation of the aortic valve whenever possible is the established procedure. This short report emphasizes the fact that individual treatment is required when SVA need to be operated, depending on the presentation, the location and the size of the finding. Surgery may consist of simple patch closure, bilateral tunnel closure (entry and exit) or more radical operation like Bentall in case the whole aortic root should be addressed. Overall results are excellent, independently of the clinical presentation (acute or elective) with a mortality approaching zero.

Cardiac defects of hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders: a retrospective cohort study.

Defective connective tissue structure may cause individuals with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSD) to develop cardiac defects. We conducted a retrospective chart review of adult patients treated in the EDS Clinic from November 1, 2019, to June 20, 2022 to identify those with cardiac defects. Echocardiogram data were collected using a data collection service. All EDS Clinic patients were evaluated by a single physician and diagnosed according to the 2017 EDS diagnostic criteria. Patient demographic, family and cardiac history were extracted from self-reported responses from a REDCap clinical intake questionnaire. Patients with at least 1 available echocardiogram (ECHO) were selected for the study (n = 568). The prevalence of aortic root dilation in patients with hEDS was 2.7% and for HSD was 0.6%, with larger measurements for males than females and with age. Based on self-reported cardiac history that was verified from the medical record, patients with hEDS with bradycardia (p = 0.034) or brain aneurysm (p = 0.015) had a significantly larger average adult aortic root z-score. In contrast, patients with HSD that self-reported dysautonomia (p = 0.019) had a significantly larger average aortic root z-score. The prevalence of diagnosed mitral valve prolapse in patients with hEDS was 3.5% and HSD was 1.8%. Variants of uncertain significance were identified in 16 of 84 patients that received genetic testing based on family history. These data reveal a low prevalence of cardiac defects in a large cohort of well-characterized hEDS and HSD patients. Differences in cardiovascular issues were not observed between patients with hEDS vs. HSD; and our findings suggest that cardiac defects in patients with hEDS or HSD are similar to the general population.

Association of pulse pressure and aortic root diameter in elderly Chinese patients with chronic heart failure.

High pulse pressure (PP) and aortic root diameter (AoD) are hallmarks of arterial stiffness or vascular aging and they are considered as risk factors for age-related cardiovascular disease, including heart failure (HF). However, the relationship between PP and AoD in patients with heart failure (HF) is uncertain. This study aimed to evaluate the relationship between PP and AoD in the middle-aged and the elderly with HF. A total of 1,027 Chinese middle-aged and elderly patients with HF, including HF with reduced ejection fraction (HFrEF), HF with mid-range EF (HFmrEF), and HF with preserved EF (HFpEF) were included in this study. Pearson correlation analysis was used to evaluate the relationship between PP and AoD in the three types of HF. Multiple linear regression analysis was performed to assess the factors that affected AoD. Multivariate logistic regression was performed to determine the association between the PP level quartiles and AoD. The results were validated in an independent dataset included a total of 378 consecutive patients with HFrEF hospitalized at the Pingtan Branch of Fujian Medical University Union Hospital (Fujian, China). There was a positive correlation between PP and AoD in the middle-aged and the elderly with HFrEF. Multiple linear regression analysis revealed that PP, age, and body mass index (BMI) were independently correlated with AoD in HFrEF patients. In multivariate logistic regression analysis, an increased risk of aortic root dilation was observed in the highest quartile of the PP level compared with the lowest quartile. Age significantly interacted with PP (p = 0.047). A significant association between PP levels and AoD was only observed in patients ≥ 65 years old, but not in patients < 65 years old. In the validation dataset, PP was independently related to AoD in patients with HFrEF (β = 0.205, p = 0.001). PP level was independently and positively associated with AoD, especially in the elderly with HFrEF, but not in patients with HFmrEF and HFpEF. Arterial stiffening or vascular aging may play a certain role in the elderly HFrEF patients.

Segmental analysis of aortic basal ring dimensions in normal and dilated tricuspid aortic roots.

In patients with aortic root aneurysm, the aortic basal ring is frequently dilated. It has been speculated that the muscular part of the basal ring dilates most. The purpose of this study was to analyse the segmental dilatation of the basal ring, comparing normal and dilated roots in patients with tricuspid aortic valves. Retrospective analysis of computed tomography studies in patients with normal and dilated aortic roots was performed. Lengths of segments of the basal ring corresponding to each of the 3 sinuses, and to the muscular and fibrous parts were measured. Fractions of these segments relative to the total basal ring perimeter were calculated. We analysed 152 normal and 126 dilated aortic roots and 86 propensity-matched pairs. Basal ring dilatation was present in all segments of dilated aortic roots with subtle differences between the segments corresponding to the 3 sinuses. The muscular part of the basal ring dilated proportionately to its fibrous part, with no difference in fractions of measured muscular part in normal and dilated roots [42.2% (interquartile range 4.3%) vs 42.1% (interquartile range 6.3%)]. Basal ring dilatation was present in all segments corresponding to the 3 sinuses in dilated aortic roots. Both muscular and fibrous parts dilated equally, supporting the need to stabilize the entire basal ring when performing aortic valve repair surgery.