Sinus Of Valsalva Aneurysm Research Articles

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 >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 (<4cm), mild (4.0-4.5cm), moderate (4.5-5cm) and severely (>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<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<0.001), STJ 39% (HR 2.82; CI 1.64-4.86; p<0.001) and AscAo 44% (HR 4.74; CI 2.46-9.13; p<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.

Association of lifelong exercise characteristics with aortic pathology in patients with Marfan syndrome

Abstract Background Marfan syndrome (MFS) is a genetic connective tissue disease with an increased risk for aortic root dilatation and aortic dissection. Current guidelines, based on expert consensus, recommend exercise restrictions due to concerns that exercise-induced hemodynamic stress may accelerate aortopathy. Studies examining exercise characteristics and their association with aortopathy in MFS patients are lacking. Purpose To assess the association of lifelong exercise characteristics (volume, intensity and type) with the presence of aortic dilatation in MFS patients. Methods An international multi-centre study was conducted in MFS adults. Patients were classified as sedentary (<500 metabolic equivalent of task minutes per week [MET-min/wk]), active (500-1000 MET-min/wk), or highly active (≥1000 MET-min/wk) based on lifelong exercise volumes assessed by a validated questionnaire. Patient’s dominant exercise intensity was classified as moderate (3-6 MET) or vigorous (≥6 MET), and sport type as skill, power, mixed, or endurance. Echocardiography was used to assess diameters at the sinuses of Valsalva (SoV) and ascending aorta (AA), and Campens Z-scores were calculated. Aortic dilatation was evaluated as a diameter ≥40, ≥45 mm, a Z-score ≥2 or ≥3 respectively. Results 315 patients (36[27-49] years, 53% male) were included of which 104 were sedentary, 77 active and 134 highly active (Fig. 1). 42 patients did not perform sport-related exercise, whereas 147 and 121 patients predominantly participated in moderate or vigorous intensity sports. Patients performing sports were allocated to the skill (n=13), power (n=27), mixed (n=158), endurance (n=66) or non-dominant (n=9) exercise type group. Mean SoV and AA diameters were 42.9±7.7 mm and 33.9±7.3 mm. SoV or AA diameters of ≥40 mm were present in 200 (64%) and 41 (13%) patients, whereas 223 (71%) and 81 (26%) patients had a Z-score ≥2 for the SoV or AA. Exercise volume was not associated with SoV dilatation (Fig. 2). Moderate and vigorous intensity sports were associated with a lower prevalence of SoV Z-score ≥3. Power sport was associated with a lower prevalence of all definitions of SoV dilatation, whereas mixed and endurance sports were associated with a lower prevalence of SoV Z-score ≥3. No associations were found with AA dilatation. Of the 17 (5%) patients who suffered from an aortic dissection, 1 case occurred during walking (type A dissection) whereas all other cases were not exercise-related. Conclusions Lifelong exercise volumes were not associated with aortic dilatation in MFS patients. Power sports and vigorous intensity exercise were inversely related to SoV dilatation. Further investigation is warranted to clarify whether this association reflects that patients engaging in these activities have a less severe cardiovascular phenotype, or that they are protected from exercise-related aortic complications, and if patients may miss health benefits due to too exercise restrictions.

Higher left ventricular stroke volume is associated with aortic dilatation in repaired tetralogy of Fallot patients.

Aortic root dilation has been reported commonly after repair of tetralogy of Fallot. However, the rate and risk factors of progression of the dilation are not fully understood. This is a single-centre, retrospective study to assess the rate and factors associated with progressive dilatation of the aortic root in repaired tetralogy of Fallot patients using cardiac MRI. The presence of the significant aortic dilation and the progression of dilation between initial and follow-up cardiac MRI were examined. The study cohort comprised 72 patients with repaired tetralogy of Fallot. The median age at the initial cardiac MRI scan was 19.6 (interquartile range: 14.6-31) years, and the median follow-up interval was 4.3 (2.9-5.7) years. Median dimension of ascending aorta at initial and follow-up cardiac MRI was 27.0 (22.3-31.0) mm and 29.2 (25.0-32.1) mm, respectively. Significant aortic dilation (the percentage predicted ascending aorta ≥150%) was observed in 11 (15.2%) patients at the initial cardiac MRI and 24 (33.3%) at the follow-up cardiac MRI. The significant aortic dilation at follow-up cardiac MRI was associated with increased indexed left ventricular stroke volume (odds ratio 1.062, p = 0.023). Thirteen patients demonstrated the significant progressive dilation of aorta between initial and follow-up cardiac MRI. The progressive dilation was associated with left ventricular ejection fraction at initial cardiac MRI (odds ratio 1.135, p = 0.048). In patients with repaired tetralogy of Fallot, aortic dilation is common and progresses over time. Cardiac MRI is a valuable tool for identifying individuals at risk for progressive aortic dilation.

Evaluation of the clinical features of an outpatient cohort with Marfan syndrome

Marfan syndrome is a connective tissue disease with autosomal dominant inheritance and variable clinical presentation. The main clinical manifestations recognition could contribute to early diagnosis and cardiovascular complication prevention. We aimed to evaluate the clinical profile of a Marfan syndrome outpatient cohort. MethodsRetrospective cross-sectional study was carried out with outpatients over 12 years of age whose electronic medical records contained the clinical information and complementary exams necessary for study inclusion. Data were analyzed using descriptive statistics and comparisons were performed using student's t-test and chi-square or Fisher's exact test. P-values<0.05 were considered statistically significant. Results75 patients (29.5 ± 13.4 years) were included and 43(57 %) were female. Positive family history for the syndrome was observed in 55(73 %) patients and ectopia lentis in 37(49 %). Positive systemic score (≥7) was identified in 60(80 %) individuals and the most frequent score components were: skin striae in 64(85 %), scoliosis in 59(79 %), wrist and thumb sign in 45(60 %), moderate or severe myopia in 43(57 %) and plain flat foot in 40(53 %). Cardiovascular symptoms occurred in 17(23 %) patients: dyspnea in 10(13 %) and palpitations in 6(8 %). Mitral valve prolapse was observed in 32(43 %) participants and aortic root dilation (z-score ≥ 2) in 53(71 %), without significant difference between the groups with or without these alterations concerning sex, age, or symptom presence. ConclusionClinical profile of a Marfan syndrome outpatient cohort includes adolescents and young adults, most without cardiovascular symptoms and with a high incidence of skeletal, ophthalmological, and cardiovascular involvement. Recognizing these clinical signs could contribute to early disease diagnosis in the general population.

Aortic valve replacement in younger patients with aortic valve insuffienciency

Aortic valve replacement is the common treatment of choice for severe aortic valve insufficiency (AI). AI is often caused by aortic root dilatation which leads to valve incompetence despite structurally normal valve leaflets. For this condition, aortic repair surgery with ring annuloplasty is considered a potentially superior option. Despite the availability of different annuloplasty rings, no guidelines for the surgical procedure nor the choice of annuloplasty ring exist. In this review, we outline the surgical treatment using valve-preserving procedures as an alternative to conventional aortic valve replacement.

Aortic regurgitation in left ventricular assist device patients - Does aortic root dilatation contribute to valve incompetence?

Aortic regurgitation (AR) is a known complication after left ventricular assist device (LVAD) implantation potentially leading to recurrent heart failure. Possible pathomechanisms include valvular pathologies and aortic root dilatation. We assessed aortic root dimensions in a group of consecutive LVAD patients who received HeartMate 3. Since 11/2015, we identified 68 patients with no or mild AR at the time of HeartMate 3 implantation who underwent serial echocardiography to assess AR and aortic root dimensions (annulus, sinus, and sinotubular junction). Median follow-up was 40 months (2-94 months). Results were correlated with clinical outcomes. Patients were 60 ± 10 years old, predominantly male (88%) and 35% presented in preoperative critical condition as defined by INTERMACS levels 1 and 2. During follow-up, 23 patients developed AR ≥ II (34%). Actuarial incidence was 8% at 1 year, 29% at 3 years and 41% at 5 years. Echocardiography revealed practically stable root dimensions at the latest follow-up compared to the preoperative state (annulus: 23 ± 3 mm vs. 23 ± 2 mm, sinus: 32 ± 4 mm vs. 33 ± 3 mm, sinotubular junction: 27 ± 3 mm vs. 28 ± 3 mm), irrespective of the development of AR. Serial CT angiograms were performed in 13 patients to confirm echocardiographic findings. Twenty-one patients died during LVAD support leading to a 5-year survival of 71%, showing no difference between patients with and without AR ≥ II (p = 0.573). At least moderate AR develops over time in a substantial fraction of patients (one-third over 3 years). The mechanism does not seem to be related to dilatation of the aortic annulus or root.

Magnetic Resonance Imaging of Temporomandibular Joint and Aortic Root Score in Fibrillinopathies.

Background and Objectives: Fibrillinopathies have different phenotypic expression: Marfan-like skeleton features, MLSF; mitral valve prolapse syndrome, MVPS; MASS phenotype, M = mitral valve prolapse, MVP, A = aortic root dilation, S = skeletal features, and the second S = (cutaneous) striae; Marfan Syndrome, MS. The study had the following main objectives: the correlation between disc displacement, DD (a major sign for temporomandibular joint dysfunction, TMDs) on magnetic resonance imaging, MRI, and aortic Z score (a major sign for aortic root dilation) on echocardiography; the predictive value of DD towards aortic Z score. DD had 2 types of severity: DD with reduction (the mild type, DDwR), and DD without reduction (the severe type, DDwoR). Materials and Methods: The type of fibrillinopathy was precised by clinical exam (systemic score), ophthalmic exam (for ectopia lentis), and echocardiography (aortic Z score, MVP). Medical treatment consisted of betablockers, BB (for patients with tachycardia), or angiotensin II receptor blockers, ARB. Surgical treatment was addressed to aortic root aneurysm, and severe mitral regurgitation, MR. DD benefited from dental conservatory treatment or surgical restoration. Results: DD-Z score had a powerful correlation in MASS (Rho = 0.787, p < 0.01), and in MS patients (Rho = 0.819, p < 0.01). For the entire sample, both DDwR-Z score and DDwoR-Z score had a moderate correlation (Rho = 0.469, and respectively 0.669). Furthermore, DD was an important predictor for Z score. DDwoR had a double power of prediction for the Z score compared with DDwR (B coefficient = 1.661 for DDwR and 3.281 for DDwoR). Conclusions: TMDs had a powerful correlation with aortic root dilation in MASS and MS patients from the sample. Likewise, TMDs was a major predictor for aortic root dilation, in the entire sample. In clinical practice, we can utilize an extracardiac finding, TMDs, obtained by a non-invasive technique, MRI, for cardiac severity stratification of fibrillinopathies.

Aortic Root Dilation and Genotype Associations in Phelan-McDermid Syndrome.

Phelan-McDermid syndrome (PMS) is a rare genetic neurodevelopmental disorder that results from the loss of one functional copy of the SHANK3 gene. While many clinical features of PMS are well-understood, there is currently limited literature on cardiovascular abnormalities in PMS. This report aims to evaluate the prevalence of aortic root dilation (ARD) among individuals with PMS and to understand if underlying genetic variation relates to risk for ARD. We present findings from 59 participants collected from a multisite observational study evaluating the phenotype and natural history of PMS. Individual echocardiographic and genetic reports were analyzed for aortic root measurements and genetic variant data, respectively. Our a priori hypothesis was that participants with chromosome 22 deletions with hg19 start coordinates on or before 49,900,000 (larger deletions) would have more instances of ARD than participants with deletion start coordinates after 49,900,000 (smaller deletions). Eight participants (14%) had ARD, and its presence was statistically significantly associated with large deletions (p = 0.047). Relatedly, participants with ARD had significantly more genes deleted on chromosome 22 than participants without ARD (p = 0.013). These results could aid in the identification of individuals with PMS who are at higher risk for ARD.

Management of aortic disease in children with FBN1-related Marfan syndrome.

Marfan syndrome (MFS) is a hereditary connective tissue disorder with an estimated prevalence of 1:5000-1:10 000 individuals. It is a pleiotropic disease characterized by specific ocular, cardiovascular, and skeletal features. The most common cardiovascular complication is aortic root dilatation which untreated can lead to life-threatening aortic root dissection, mainly occurring in adult patients. Prompt diagnosis, appropriate follow-up, and timely treatment can prevent aortic events. Currently there are no specific recommendations for treatment of children with MFS, and management is greatly based on adult guidelines. Furthermore, due to the scarcity of studies including children, there is a lack of uniform treatment across different centres. This consensus document aims at bridging these gaps of knowledge. This work is a joint collaboration between the paediatric subgroup of the European Network of Vascular Diseases (VASCERN, Heritable Thoracic Aortic Disease Working Group) and the Association for European Paediatric and Congenital Cardiology (AEPC). A group of experts from 12 different centres and 8 different countries participated in this effort. This document reviews four main subjects, namely, (i) imaging of the aorta at diagnosis and follow-up, (ii) recommendations on medical treatment, (iii) recommendations on surgical treatment, and (iv) recommendations on sport participation.

Ruptured coronary sinus aneurysm in pregnancy combined with cardiogenic shock and elevated V1 and aVR ST segments: Case report.

Sinus of Valsalva aneurysm (SOVA), a rare cardiac malformation, is usually congenital and rarely acquired and most commonly occurring in the right coronary sinus. The clinical presentation of patients with SOVA varies. It is usually asymptomatic when it has not ruptured, and when it compresses neighboring structures or ruptures, it can lead to heart failure or shock, at which point urgent surgical intervention is usually required. Rupture of the sinus of Valsalva aneurysm (RSOVA) during pregnancy is really hard to come by, especially if the clinical presentations resemble that of an acute myocardial infarction. This report describes a pregnant woman with severe chest pain and hypotension with aVR and V1 ST-segment elevation due to RSOVA. Effects of RSOVA on the fetus, disease survival, and prognosis. RSOVA. Open SOVA repair. The patient's blood pressure returned to normal range and clinical symptoms disappeared after the surgery. After 3 months of follow-up, the patient was hemodynamically stable without chest discomfort, and an echocardiogram showed a normal aortic sinus. Progressive aneurysm dilatation or rupture has a poor prognosis. A thorough history and physical examination are fundamental, with echocardiography being the initial diagnostic tool of choice, and other ancillary tests (e.g., computed tomography) being used to complement and confirm the diagnosis. Surgery remains the current treatment of choice for patients with RSOVA, while the continuation of pregnancy in pregnant patients with RSOVA remains a case-by-case measure.

Aortitis Secondary to Prosthetic Valve Endocarditis: A Rare Complication Unveiled

Abstract Introduction Aortitis includes both infectious and non-infectious inflammation of the arterial wall. Infectious forms have a worse prognosis, necessitating immediate diagnosis and treatment. We report a case of a patient with prosthetic valve endocarditis complicated with infectious aortitis and multiple embolic lesions, ultimately leading to death. Case presentation An 81-year-old man with an aortic valve bioprosthesis (Intuity 27 mm) presented with fever, dysuria, and ankle swelling. Laboratory showed leukocytosis, anemia, elevated NT-proBNP, and C-reactive protein. Echocardiography revealed a hyperechogenic mass on the bioprosthesis, confirmed as prosthetic endocarditis by transesophageal echocardiography, which also showed an aortic bulb aneurysm and a 9 mm arterial wall thickening. Blood cultures were positive for Staphylococcus aureus, and intravenous antibiotics were started. Thoracic computed tomography confirmed aortic root dilation and mural thickening, indicating infectious aortitis with multiple embolic lesions in the spleen and kidneys. The patient then suffered an intestinal sub-occlusion. The Heart Team decided against cardiovascular surgery due to frailty and high surgical risk. Despite initial hemodynamic stability, his condition deteriorated, leading to death. Conclusion This case presents a challenging scenario of prosthetic valvular endocarditis complicated by infectious aortitis, aortic aneurysm, and multiple thromboembolic lesions in a high-risk patient, ultimately resulting in death.

Bicuspid Aortic Valve Disease: From Pathophysiology to Treatment.

The Bicuspid Aortic Valve (BAV) is the most common congenital anomaly in adults, with a global incidence of 1.3%. Despite being well documented, BAV presents significant clinical challenges due to its phenotypic heterogeneity, diverse clinical manifestations, and variable outcomes. Pathophysiologically, BAV differs from tricuspid valves in calcification patterns and hemodynamic effects, leading to increased shear stress and aortic root dilatation, while it is influenced by genetic and hemodynamic factors. This is why therapeutically, BAV presents challenges for both surgical and transcatheter interventions, with surgical approaches being traditionally preferred, especially when aortopathy is present. However, transcatheter aortic valve implantation (TAVI) has emerged as a viable option, with studies showing comparable outcomes to surgery in selected patients, while advancements in TAVI and a better understanding of BAV's genetic and pathophysiological nuances are expanding treatment options. The choice between mechanical and bioprosthetic valves also presents considerations, particularly regarding long-term durability and the need for anticoagulation. Future research should focus on long-term registries and genetic studies to refine therapeutic strategies and improve patient outcomes. This review aims to evaluate current approaches in the surgical and interventional management of BAV, focusing on its anatomy, pathogenesis, pathophysiology, and therapeutic strategies.

Longitudinal echocardiography in pediatric patients with hypermobile Ehlers-Danlos syndrome.

Vascular Ehlers-Danlos, Marfan and Loeys-Dietz syndromes have increased risk of aortic dilation and dissection. Previous early studies showed hypermobile Ehlers-Danlos syndrome (hEDS) may also have increased risk, with echocardiography screening recommended; subsequent studies have not confirmed the risk or recommended echocardiography. This pediatric-based study assessed aortic dilation prevalence in those with hEDS by serial echocardiographic examinations and assessed family history for aortic dissections. We retrospectively identified individuals with hEDS who had echocardiography studies from the electronic medical records at one pediatric center. Aortic root Z-scores >2.0 were found in 15/225 subjects (average age 12.9 years) on initial echocardiograms, with no Z-score >3.0. Subsequent studies (n = 68) found statistically significant decline in aortic root Z-scores. Repeat echocardiography in those with initial aortic root Z-score >2.0 (n = 10) demonstrated a decline in Z score <2.0 in seven. On final examination, 9/225 (4.0%) had a Z-score >2.0, not statistically different from the general population. No aortic dissection occurred in first- or second-degree relatives. In conclusion, aortic root dilation rate in hEDS is likely not different from the general population. We propose that in the absence of other cardiac findings or suspicion for another disorder, echocardiography is not required in hEDS.

Development of a Simple Analytical Model to Facilitate Preoperative Surgical Planning in Valve-Sparing Aortic Root Replacement.

Valve-sparing root replacement (VSRR) is attractive for aortic root dilation as it preserves the native aortic valve (AoV). Low effective height (eH) after reconstruction is a risk factor for repair failure and reoperation. We developed and validated a quantitative AoV repair strategy to reliably restore normal valve proportions to promote long-term function. Normal AoV proportions were used to derive geometric relationships for sinotubular junction diameter (DSTJ), free edge length (FEL), free edge angle, and commissure height. These relationships informed two models for predicting eH following VSRR: (1) assuming valve symmetry and (2) accounting for valve asymmetry. Porcine heart (n = 6) ex vivo validation was performed under 4 VSRR scenarios: "Ideal" (tube graft size targeting FEL/DSTJ = 1.28), "Oversized" (one graft size larger than Ideal), "Undersized" (two sizes smaller), and "Undersized + Plicated" (FEL/DSTJ = 1.28 restored with leaflet plication). Our analytical models predicted eH using preoperative measurements and estimated reconstructed dimensions. The Oversized graft exhibited similar eH to Ideal but higher regurgitation in the ex vivo model, whereas the Undersized graft demonstrated lower eH and regurgitation. Plication in the Undersized graft restored valve function (regurgitation & eH) similar to Ideal in the ex vivo model and above Ideal in the analytical models. Both analytical models predicted ex vivo eH well except in the Oversized and Undersized + Plicated conditions. Utilizing measurements from preoperative imaging and simple mathematical models, patient-specific operative plans for VSRR can be created by estimating valve dimensions necessary to achieve favorable valve features post-repair. Clinical application of this approach promises to improve consistency in achieving optimal long-term dimensions and durability.

366 A Case Study: Application of Personalised External Aortic Root Support to Overcome a Failing Ross Procedure

Abstract Aortic root aneurysms are traditionally treated by aortic root replacement surgery alone or in combination with aortic valve replacement in a Modified Bentall procedure. We present a novel surgical approach using a Personalised External Aortic Root Support (PEARS) in the management of an aortic root aneurysm in a 24-year-old male, initially presenting in infancy with mixed aortic valve disease who underwent a free root Ross procedure in 2010 aged 11. Follow-up cardiac magnetic resonance imaging and transthoracic echocardiography showed mild aortic and pulmonary valve regurgitation with aortic root dilatation (51mm). In 2022, Computerised tomography (CT) exhibited dilation of the Sinus of Valsalva (43mm) and ascending aorta (56 mm). MDT review deemed redo valve-sparing root replacement appropriate as the ascending aorta was &amp;gt;55mm with an approximate 8mm increase over three years. As the young patient wished to avoid warfarinisation and the autograft valve maintained good function, a reduction PEARS was considered. In September 2023, a reduced aortic PEARS graft (80% reduction) was implemented along with a redo of the right ventricle to pulmonary artery conduit. Whilst applying the PEARS it was noted that there was a retroaortic circumflex artery, care was taken to mobilise this aberrant vessel to avoid compression by the graft. Cardiopulmonary bypass and cross-clamp times were 180 and 116 minutes respectively. Intraoperative transoesophageal echocardiography confirmed good bi-ventricular function with no pulmonary regurgitation additionally on postoperative CT the aortic diameter was 38mm. In conclusion, application of a reduction PEARS is a potential alternative to a valve-sparing aortic root replacement method in young patients.

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.