Successful Balloon Valvuloplasty in a Case of Neonatal Aortic Critical Stenosis With Bicuspid Aortic Valve (BAV): A Case Report.

A bicuspid aortic valve (BAV) refers to a condition in which the valve has two cusps rather than three. Usually ignored, this manifests itself later in life. This bicuspid valve may manifest earlier in children with significant aortic stenosis because they have a severe left ventricular outflow tract restriction that worsens over time. This syndrome commonly results in congestive heart failure in newborns and early neonatal life. There can be a small amount of significant risk in pediatric patients having this BAV causing stenosis and being one of the reasons for sudden, unexpected death. Morphological differences result from a congenital cardiac abnormality called BAV. This paper emphasizes the importance of a multidisciplinary team in managing BAV and critical aortic stenosis and provides evaluation and treatment guidelines for both conditions. Transcatheter or surgical intervention is used for symptomatic individuals or those with a moderate to severe obstruction of the left ventricular outflow tract.

Aortic valve function and aortic dimensions in obese and overweight patients with bicuspid aortic valve.

INTRODUCTION: Bicuspid aortic valve is the most common CHD and commonly associated with activity restrictions that may lead to a sedentary lifestyle known to increase obesity risk. It is unknown whether obesity is associated with changes in aortic dimensions or aortic valve function in young people with bicuspid aortic valve. This study investigates whether overweight and obese children with bicuspid aortic valve have worse aortic valve function or increased aortic dimensions compared to healthy weight children with bicuspid aortic valve. METHODS: This was a single centre retrospective cohort study comprised of patients 5 to 25 years old with a diagnosis of bicuspid aortic valve between 1 January, 2019 and 31 December, 2020. Patients were classified as healthy weight or overweight/obese. Values for aortic dimensions as well as peak and mean aortic valve gradients were obtained from echocardiogram reports. RESULTS: About 251 patients were analysed. Demographics were similar between groups. When indexed to height, the aortic valve annulus (1.28 ± 0.14 vs. 1.34 ± 0.15, p = 0.001) and sinotubular junctions (1.44 ± 0.21 vs. 1.49 ± 0.24, p = 0.038) were larger in the overweight/obese group, with no differences in aortic root or ascending aorta sizes. The obese/overweight group had a higher peak aortic valve gradient (23.03 ± 1.64 mmHg vs. 16.17 ± 1.55 mmHg, p = 0.003) compared to the healthy weight group. CONCLUSION: Healthy weight patients did not have larger aortic dimensions compared to the overweight/obese patients. There was evidence of worsening aortic valve stenosis in overweight/obese patients compared to those at a healthy weight.

Aortic root replacement for bicuspid aortic valve dysfunction does not impair survival rates.

BACKGROUND: Patients with a bicuspid aortic valve carry an increased risk for developing an ascending aortic aneurysm due to intrinsic aortic wall alterations. A lower threshold for aortic surgery may therefore be considered in these patients, especially in those who require aortic valve surgery. This study aimed to compare the outcomes of an isolated aortic valve replacement with that of an aortic root replacement in bicuspid aortic valve patients with an indication for aortic valve surgery. METHODS: Patients were included in retrospect from a tertiary academic hospital. Included patients received an elective aortic valve (AVR) or a composite valve-graft conduit (both mechanical and biological) between 2006 and 2021 without any concomitant procedure. Mortality data were retrieved from a national database and comparisons, including survival analyses, were performed between both groups. RESULTS: A total of 132 isolated AVR and 149 aortic root replacements were included. Patients who received an isolated AVR were significantly older than the aortic root replacement group (62.9 vs. 57.7 year respectively, p < 0.001). Survival analyses showed a comparable long-term mortality between both groups (8.1% vs. 9.1%, p = 0.321). CONCLUSION: This study shows that performing an aortic root replacement with a composite valve-graft conduit in bicuspid aortic valve patients does not impair the survival outcomes. In the light of preventing potential future aortic complications within this patient group with a congenitally and structurally weakened aortic wall, a more aggressive approach towards the treatment of BAV aortopathy might be considered.

Physiological Insights From Asymptomatic Severe Valvular Disease in a Highly Trained Athlete.

Parameters relied on as tools for prognostication in valvular disease can be confounded by athletic physiological remodeling. This case describes how cardiopulmonary exercise testing and multimodality imaging may be helpful in assessment of a 46-year-old female athlete with bicuspid aortic valve and subaortic membrane with associated asymptomatic severe mixed aortic valve disease.

Unraveling aortic hemodynamics using fluid structure interaction: biomechanical insights into bicuspid aortic valve dynamics with multiple aortic lesions.

Aortic lesions, exemplified by bicuspid aortic valves (BAVs), can complicate congenital heart defects, particularly in Turner syndrome patients. The combination of BAV, dilated ascending aorta, and an elongated aortic arch presents complex hemodynamics, requiring detailed analysis for tailored treatment strategies. While current clinical decision-making relies on imaging modalities offering limited biomechanical insights, integrating high-performance computing and fluid-structure interaction algorithms with patient data enables comprehensive evaluation of diseased anatomy and planned intervention. In this study, a patient-specific workflow was utilized to biomechanically assess a Turner syndrome patient's BAV, dilated ascending aorta, and elongated arch. Results showed significant improvements in valve function (effective orifice area, EOA increased approximately twofold) and reduction in valve stress (~ 1.8-fold) following virtual commissurotomy, leading to enhanced flow dynamics and decreased viscous dissipation (~ twofold) particularly in the ascending aorta. However, increased viscous dissipation in the distal transverse aortic arch offset its local reduction in the AAo post-intervention, emphasizing the elongated arch's role in aortic hemodynamics. Our findings highlight the importance of comprehensive biomechanical evaluation and integrating patient-specific modeling with conventional imaging techniques for improved disease assessment, risk stratification, and treatment planning, ultimately enhancing patient outcomes.

RATE OF ASCENDING AORTIC ENLARGEMENT IN A LARGE ECHOCARDIOGRAPHIC COHORT: ASSOCIATED RISK FACTORS AND ADVERSE AORTIC EVENTS.

BACKGROUND: Understanding ascending aortic aneurysm growth and associated risk factors is critical to advising appropriate echocardiographic follow-up intervals for patients. This study aimed to identify aortic aneurysm growth rate on serial echocardiograms as well as the clinical and demographic variables that contribute to baseline aortic size and subsequent aortic growth. METHODS: Patients identified with ascending aortic aneurysm and undergoing serial echocardiograms within five years were evaluated. Ascending aortic size was measured as part of routine echocardiogram exams. Clinical and demographic variables including aortic valve type (trileaflet, bicuspid or prosthetic) were evaluated for association with baseline aortic size as well as with aortic progression rate. Clinical events including aortic dissection, elective or emergent surgical repair were recorded. RESULTS: 3639 patients were identified, (78% males, median age of 69 years, 175 (4.8%) with bicuspid valve, and 206 (5.6%) with prior aortic valve replacement (AVR). Patients with larger aorta at baseline were older with higher tobacco use and prior prosthetic valves. Over a mean of 2.4 years, aortic growth was observed and differed by valve type (trileaflet valve: 0.08 mm/year , bicuspid valve: 0.4 mm/year , p<0.001). In six patients who developed aortic dissection the estimated average annual growth rate was 0.98 mm/year. CONCLUSIONS: In a large echo cohort, aortic aneurysm growth rate was 0.08 mm/year though higher in those with bicuspid valves ( 0.4 mm/ year), but initial aortic size did not correlate with change in the aortic progression rate. This data may help inform recommended echocardiographic surveillance intervals.

Circulating Matrix Metalloproteinases for Prediction of Aortic Dilatation in Children with Bicuspid Aortic Valve: A Single-Center, Observational Study.

Circulating biomarkers have been proposed for early identification of aortic dilatation progression associated with bicuspid aortic valve (BAV), but matrix metalloproteinases (MMPs) are distinguished as signatures of increased extracellular matrix degradation, a landmark of aneurysm formation. The current study aims to identify the role of MMP-1, MMP-2, MMP-9, and the MMP inhibitor, TIMP-1, in identifying aortic dilation in children with BAV. We conducted a study on 73 children divided into two study groups, depending on the presence of aortic dilatation (group 1-43 BAV controls and group 2-30 children with BAV and aortic dilatation). Each patient underwent a cardiac ultrasound and, in each case, serum MMP-1, MMP-2, MMP-9, and TIMP-1 were quantified using xMAP technology. Comparison of the MMPs between the two study groups revealed significantly higher values only in the case of TIMP-1, among BAV controls. Moreover, the same TIMP-1 inversely correlated with aortic annulus absolute size and z score, as well as with ascending aorta z score. No particular correlation between the aortic phenotype and the presence of aortic dilatation was found. Future longitudinal research starting at pediatric ages could show the significance of MMPs screening in BAV individuals as predictors of aortic aneurysm formation.

Bicuspid Aortic Valve in Children and Young Adults for Cardiologists and Cardiac Surgeons: State-of-the-Art of Literature Review.

Bicuspid aortic valve disease is the most prevalent congenital heart disease, affecting up to 2% of the general population. The presentation of symptoms may vary based on the patient's anatomy of fusion, with transthoracic echocardiography being the primary diagnostic tool. Bicuspid aortic valves may also appear with concomitant aortopathy, featuring fundamental structural changes which can lead to valve dysfunction and/or aortic dilatation over time. This article seeks to give a comprehensive overview of the presentation, treatment possibilities and long-term effects of this condition. The databases MEDLINE, Embase, and the Cochrane Library were searched using the terms "endocarditis" or "bicuspid aortic valve" in combination with "epidemiology", "pathogenesis", "manifestations", "imaging", "treatment", or "surgery" to retrieve relevant articles. We have identified two types of bicuspid aortic valve disease: aortic stenosis and aortic regurgitation. Valve replacement or repair is often necessary. Patients need to be informed about the benefits and drawbacks of different valve substitutes, particularly with regard to life-long anticoagulation and female patients of childbearing age. Depending on the expertise of the surgeon and institution, the Ross procedure may be a viable alternative. Management of these patients should take into account the likelihood of somatic growth, risk of re-intervention, and anticoagulation risks that are specific to the patient, alongside the expertise of the surgeon or centre. Further research is required on the secondary prevention of patients with bicuspid aortic valve (BAV), such as lifestyle advice and antibiotics to prevent infections, as the guidelines are unclear and lack strong evidence.

Bicuspid Valve Aortopathy: Is It Reasonable to Define a Different Surgical Cutoff Based on Different Aortic Wall Mechanical Properties Compared to Those of the Tricuspid Valve?

BACKGROUND: In this study, we examined and compared ex vivo mechanical properties of aortic walls in patients with bicuspid (BAV) and tricuspid (TAV) aortic valve aortopathy to investigate if the anatomical peculiarities in the BAV group are related to an increased frailty of the aortic wall and, therefore, if a different surgical cutoff point for ascending aortic replacement could be reasonable in such patients. METHODS: Ultimate stress tests were performed on fresh aortic wall specimens harvested during elective aortic surgery in BAV (n. 33) and TAV (n. 77) patients. Three mechanical parameters were evaluated at the failure point, under both longitudinal and circumferential forces: the peak strain (Pstr), peak stress (PS), and maximum elastic modulus (EM). The relationships between the three mechanical parameters and preoperative characteristics were evaluated, with a special focus on evaluating potential risk factors for severely impaired mechanical properties, cumulatively and comparatively (BAV vs. TAV groups). RESULTS: The patient populations were inhomogeneous, as BAV patients reached surgical indication, according to the maximum aortic dilatation, at a younger age (58 ± 15 vs. 64 ± 13; p = 0.0294). The extent of the maximum aortic dilatation was, conversely, similar in the two groups (52 ± 4 vs. 54 ± 7; p = 0.2331), as well as the incidences of different phenotypes of aortic dilatation (with the ascending aorta phenotype being the most frequent in 81% and 66% of the BAV and TAV patients, respectively (p = 0.1134). Cumulatively, the mechanical properties of the aortic wall were influenced mainly by the orientation of the force applied, as both PS and EM were impaired under longitudinal stress. An age of >66 and a maximum dilatation of >52 mm were shown to predict severe Pstr reduction in the overall population. Comparative analysis revealed a trend of increased mechanical properties in the BAV group, regardless of the position, the force orientation, and the phenotype of the aortic dilatation. CONCLUSIONS: BAV aortopathy is not correlated with impaired mechanical properties of the aortic wall as such. Different surgical cutoff points for BAV aortopathy, therefore, seem to be unjustified. An age of >66 and a maximum aortic dilatation of >52 mm, however, seem to significantly influence the mechanical properties of the aortic wall in both groups. These findings, therefore, could suggest the need for more accurate monitoring and evaluation in such conditions.

Early surgical outcomes of rapid deployment aortic valve replacement in bicuspid aortic valves.

Background: Biological valve prostheses in rapid-deployment aortic valve replacement (RD AVR) procedures have demonstrated excellent outcomes. However, previous studies indicate a lack of specific surgical outcomes for the bicuspid aortic valve (BAV) or recommend RD AVR implementation. The existing literature comparing the clinical and hemodynamic outcomes of patients with BAV and those with tricuspid aortic valves (TAVs) after RD AVR is limited. Therefore, this study evaluated the safety and feasibility of RD AVR in BAV, including Sievers type 0, and compared the early clinical and hemodynamic outcomes of patients who underwent RD AVR at a single center based on aortic valve type. Methods: Our institution officially commenced performing RD AVR using Intuity valves in September 2022. The medical records of 80 patients who underwent aortic valve replacement (AVR) between September 1, 2022, and July 31, 2023 were retrospectively analyzed. In this set, 30 patients underwent RD AVR for aortic stenosis. Among the 30 RD AVR cases, groups A and B comprised 16 (53.3%) patients with TAV and 14 (46.7%) with BAV, respectively. The preoperative characteristics and postoperative echocardiographic data were compared between the two groups. Results: No statistically significant differences in preoperative characteristics, including mean age and sex distribution, were found between groups A and B. Notably, no patient in both groups exhibited mild- or higher-grade aortic regurgitation. The postoperative transvalvular mean pressure gradients showed significantly lower values in group B than in group A (12.20±4.64 vs. 16.26±5.49 mmHg, P=0.03). The necessity to insert a permanent pacemaker was not found in any of the patients (0%) in group A but was found in one (7.1%) patient in group B (P=0.46). Among the BAV cases, six (20%) were categorized as Sievers type 0. Of the 14 patients in group B, six with Sievers type 0 and the remaining eight with other bicuspid valve types were designated as groups B0 and B1, respectively. Similarly, no significant difference in postoperative transvalvular mean pressure gradient was observed between the two groups (11.33±4.49 vs. 12.86±4.94 mmHg, P=0.56). No in-hospital mortality was observed among all 30 patients. Conclusions: In this study, RD AVR was considered feasible in a small, carefully selected cohort of patients with aortic stenosis, even in BAV, including Sievers type 0, as observed from the standpoint of postoperative hemodynamic outcomes and the incidence of aortic regurgitation.

Current Evidence of Unicuspid Aortic Valve in Young Adults: a Systematic Review and Metanalysis.

Unicuspid aortic valve (UAV) is a rare congenital valvular anomaly, often misdiagnosed as the more prevalent bicuspid aortic valve (BAV). The aim of this study was to explore demographic, clinical characteristics, diagnosis, surgical options, short and long term outcomes of young adults with UAV. A systematic review and meta-analysis of literature were conducted for studies (1971-2024) including patients (≥ 14 years old) with diagnosis of UAV. Among the 2953 studies retrieved, 67 case reports, 6 case series (n=130) and 13 retrospective studies (n=918), were included in the analysis. Data from retrospective studies were aggregated using a random effects model for estimating the pooled risk ratio and mean difference. UAV is mostly unicommissural in adults (mean age 36 years old at diagnosis, 76-79% males). The most common conditions associated in UAV patients were aortic coarctation (from 3.8 to 12%), ventricular septal defect (3%) and Turner syndrome (3%). In general, the diagnosis was performed with TTE and confirmed with TEE (+/- 3D-TEE). The most common types of surgery were AVR. Dilated ascending aorta was described in 44% and 35% of retrospective studies and case reports, respectively. Concomitant ascending aorta replacement/repair was reported 38% and 27% of retrospective studies and case reports, respectively. Overall survival was reported in 3 studies, ranging from 95 to 98% at 10 years. UAV should be considered a separate entity from BAV. Further investigations with regards to the possibility of a familial incidence, associated histopathological changes in the aorta, and ideal follow up and intervention are needed.

Conduction Disturbances and Outcome After Surgical Aortic Valve Replacement in Patients With Bicuspid and Tricuspid Aortic Stenosis.

BACKGROUND: This study aimed to compare the incidence and prognostic implications of new-onset conduction disturbances after surgical aortic valve replacement (SAVR) in patients with bicuspid aortic valve (BAV) aortic stenosis (AS) versus patients with tricuspid aortic valve (TAV) AS (ie, BAV-AS and TAV-AS, respectively). Additionally, the study included stratification of BAV patients according to subtype. METHODS: In this cohort study, the incidence of postoperative third-degree atrioventricular (AV) block with subsequent permanent pacemaker requirement and new-onset left bundle-branch block (LBBB) was investigated in 1147 consecutive patients without preoperative conduction disorder who underwent isolated SAVR (with or without ascending aortic surgery) between January 1, 2005, and December 31, 2022. The groups were stratified by aortic valve morphology (BAV, n=589; TAV, n=558). The outcomes of interests were new-onset third-degree AV block or new-onset LBBB during the index hospitalization. The impact of new-onset postoperative conduction disturbances on survival was investigated in BAV-AS and TAV-AS patients during a median follow-up of 8.2 years. BAV morphology was further categorized according to the Sievers and Schmidtke classification system (possible in 307 BAV-AS patients) to explore association between BAV subtypes and new-onset conduction disturbances after SAVR. RESULTS: The overall incidence of third-degree AV block and new-onset LBBB after SAVR was 4.5% and 7.8%, respectively. BAV-AS patients had a higher incidence of both new-onset third-degree AV block (6.5% versus 2.5%; P=0.001) and new-onset LBBB (9.7% versus 5.7%; P=0.013) compared with TAV-AS patients. New-onset LBBB was associated with an increased all-cause mortality during follow-up (adjusted hazard ratio, 1.60 [95% CI, 1.12-2.30]; P=0.011), whereas new-onset third-degree AV block was not associated with worse prognosis. Subgroup analysis of the BAV cohort revealed that BAV-AS patients with fusion of the right- and non-coronary cusps had the highest risk of new-onset third-degree AV block (adjusted odds ratio [aOR], 8.33 [95% CI, 3.31-20.97]; P<0.001, with TAV as reference group) and new-onset LBBB (aOR, 4.03 [95% CI, 1.84-8.82]; P<0.001, with TAV as reference group), whereas no significant association was observed for the other BAV subtypes. CONCLUSIONS: New-onset LBBB after SAVR is associated with increased all-cause mortality during follow-up, and is more frequent complication in BAV AS patients compared with TAV-AS patients. BAV-AS patients with fusion of the right- and non-coronary cusps have an increased risk for conduction disturbances after SAVR. This should be taken into consideration when managing these patients.

Deficient GATA6-CXCR7 signaling leads to bicuspid aortic valve.

The cardiac outflow tract (OFT) transiently links the ventricles to the aortic sac and forms the arterial valves. Abnormalities in these valves, such as bicuspid aortic valve (BAV), are common congenital anomalies. GATA6-inactivating variants cause cardiac OFT defects and BAV, but their mechanisms are unclear. We generated Gata6STOP/+ mice using CRISPR-Cas9, which show highly penetrant BAV (70%) and membranous ventricular septal defects (43%). These mice exhibited decreased proliferation and increased ISL1-positive progenitor cells in the OFT, indicating abnormal cardiovascular differentiation. Gata6 deletion with the Mef2cCre driver line recapitulated Gata6STOP/+ phenotypes, indicating a cell-autonomous role for Gata6 in the second heart field. Gata6STOP/+ mice showed reduced OFT length and caliber, associated with deficient cardiac neural crest cell contribution, which may cause valvulo-septal defects. RNA-sequencing analysis showed depletion in pathways related to cell proliferation and migration, highlighting Cxcr7 (also known as Ackr3) as a candidate gene. Reduced mesenchymal cell migration and invasion were observed in Gata6STOP/+ OFT tissue. CXCR7 agonists reduced mesenchymal cell migration and increased invasion in wild-type but not in Gata6STOP/+ explants, indicating the GATA6-dependent role of CXCR7 in OFT development and its potential link to BAV.

Impact of Balloon-Expandable TAVR Valve Deformation and Calcium Distribution on Outcomes in Bicuspid Aortic Valve.

BACKGROUND: Research on the role of transcatheter heart valve (THV) deformation and calcium distribution in patients with bicuspid aortic valves (BAVs) undergoing transcatheter aortic valve replacement (TAVR) remains limited. OBJECTIVES: The aim of this study was to evaluate the impact of THV deformation on clinical outcomes in individuals with BAVs undergoing TAVR and the influence of calcium on these outcomes. METHODS: In total, 229 consecutive patients with BAVs who underwent TAVR with balloon-expandable valves and had computed tomography (CT) performed 30 days post-TAVR were analyzed. Patients were stratified into 3 groups: group 1 (n = 125), with no THV underexpansion or eccentricity; group 2 (n = 69), with underexpansion or eccentricity; and group 3 (n = 35), with both. Calcium distribution was assessed using CT, and its associations with clinical outcomes, including all-cause mortality at 3 years and leaflet thrombosis at 30 days, were determined. A subgroup analysis of patients with type 1 BAVs was conducted. RESULTS: Group 3 exhibited higher rates of all-cause mortality than the other groups, along with the highest risk for hypoattenuated leaflet thickening at 30 days. Multivariate analysis identified annular and left ventricular outflow tract calcification as independent predictors of all-cause mortality and hypoattenuated leaflet thickening. In patients with type 1 BAVs, excessive calcification at the raphe and opposite leaflet were associated with all-cause mortality at 3 years. CONCLUSIONS: THV deformation post-TAVR was significantly linked to all-cause mortality in patients with BAVs. Annular and left ventricular outflow tract calcification correlated with increased risks for all-cause mortality and leaflet thrombosis. (Assessment of Transcatheter and Surgical Aortic Bioprosthetic Valve Thrombosis and Its Treatment With Anticoagulation [RESOLVE]; NCT02318342).

Predictors of left ventricular outflow tract obstruction after biventricular repair in interrupted aortic arch or aortic coarctation.

BACKGROUND: Left ventricular outflow tract obstruction (LVOTO) re-intervention is a significant cause of morbidity and mortality in patients with coarctation of the aorta (CoA) or interrupted aortic arch (IAA) after aortoplasty. METHODS: This retrospective study analyzed data from neonates with IAA/CoA who underwent biventricular repair between 2012 and 2022. LVOTO events were defined by the detection of color Doppler flow acceleration ≥3.0 m/s at the valvular, subvalvular, or supravalvular regions via transthoracic echocardiography, and the necessity for surgical or catheter intervention to relieve the obstruction. RESULTS: Among 121 neonates with CoA/IAA, 16 (13.7%) primary aortoplasty patients developed LVOTO. Additionally, one patient (25%) who underwent a staged Yasui operation developed LVOTO due to a narrowed ventricular septal defect-pulmonary atresia tunnel. During follow-up, 58% of patients with a bicuspid valve and 25% of patients with a subaortic ridge developed LVOTO. The combination of either a bicuspid valve, subaortic ridge, or an aortic valve annulus Z-score < -3.0 predicted a high re-intervention rate (7/8 [87.5%]). CONCLUSIONS: In patients with IAA/CoA, the presence of multiple risk factors, including a bicuspid valve, subaortic ridge, and an aortic valve annulus Z-score < -3.0, is associated with a significantly increased rate of re-intervention for LVOTO.

Mediastinal mayhem: the challenges of incidental thoracic arteriovenous malformations-a case report.

Background: Arteriovenous malformations (AVMs) within the mediastinum are rare vascular anomalies. With the increasing number of coronary angiographies being performed, the number of incidentally found cases is rising. This presents challenges in terms of determining the appropriate treatment strategy. Case summary: We present the case of a 79-year-old man with aortic stenosis, experiencing functional dyspnoea and fatigue. Echocardiography showed a bicuspid aortic valve, and while left heart catheterization confirmed no significant coronary stenosis, it revealed a tortuous vessel originating from the circumflex artery (Cx), assumed to be heading toward the pulmonary circulation. The patient was scheduled for a surgical replacement of the aortic valve (SAVR). During the SAVR, the tortuous vessel was revealed to be a large, complex AVM located in the mediastinum. This increased both the duration of the surgery and the use of cardioplegia. Further, bleeding occurred per-operatively. Post-operatively, the patient developed tachy-brady syndrome and was treated with a pacemaker before discharge. Discussion: Due to the rarity of incidental AVMs in the middle/posterior mediastinum, no standard treatment protocol is available. This leaves clinicians and surgeons to manage the disease on a case-by-case basis, often with limited experience to guide their decisions. This patient case underscores the challenge of determining whether patients should be offered transcatheter aortic valve implantation (TAVI) or surgery. Furthermore, it highlights the intricate challenges that can arise when dealing with thoracic AVMs during cardiac procedures, emphasizing the importance of pre-operative awareness and tailored surgical approaches based on multidisciplinary discussions.

Flow quantification within the aortic ejection tract using 4D flow cardiac MRI in patients with bicuspid aortic valve: Implications for the assessment of aortic regurgitation.

PURPOSE: The purpose of this study was to evaluate the performance of four-dimensional (4D) flow cardiac MRI in quantifying aortic flow in patients with bicuspid aortic valve (BAV). MATERIALS AND METHODS: Patients with BAV who underwent transthoracic echocardiography (TTE) and 4D flow cardiac MRI were prospectively included. Aortic flow was quantified using two-dimensional phase contrast velocimetry at the sinotubular junction and in the ascending aorta and using 4D flow in the regurgitant jet, in the left ventricular outflow tract, at the aortic annulus, the sinotubular junction, and the ascending aorta, with or without anatomical tracking. Flow quantification was compared with ventricular volumes, pulmonary flow using Pearson correlation test, bias and limits of agreement (LOA) using Bland Altman method, and with multiparametric transthoracic echocardiography quantification using weighted kappa test. RESULTS: Eighty-eight patients (63 men, 25 women) with a mean age of 50.5 ± 14.8 (standard deviation) years (age range: 20.8-78.3) were included. Changes in flow with or without tracking were modest (< 5 mL). The best correlation was obtained at the aortic annulus for forward volume (r = 0.84; LOA [-28.4; 25.3] mL) and at the regurgitant jet and sinotubular junction for regurgitant volume (r = 0.68; LOA [-27.8; 33.8] and r = 0.69; LOA [-28.6; 24.2] mL). A combined approach for regurgitant fraction and net volume calculations using forward volume measured at ANN and regurgitant volume at sinotubular junction performed better than each level taken separately (r = 0.90; LOA [-20.7; 10.0] mL and r = 0.48, LOA [-33.8; 33.4] %). The agreement between transthoracic echocardiography and 4D flow cardiac MRI for aortic regurgitation grading was poor (kappa, 0.13 to 0.42). CONCLUSION: In patients with BAV, aortic flow quantification by 4D flow cardiac MRI is the most accurate at the annulus for the forward volume, and at the sinotubular junction or directly in the jet for the regurgitant volume.

Increase in Oral Streptococcal Endocarditis Among Moderate-Risk Patients: Impact of Guideline Changes on Endocarditis Prevention.

Background: The well-established connection between oral bacteria and infective endocarditis (IE) has prompted discussions about using antibiotic prophylaxis (AP) before invasive dental procedures. In 2007/2008, guidelines restricted AP from moderate and high-risk to exclusively high-risk patients. Objectives: The authors aimed to assess whether the proportion of oral streptococcal IE increased in moderate-risk patients using University Hospital Zurich data from 2000 to 2022. Methods: Adult IE patients were categorized into risk groups based on European Society of Cardiology and Swiss guidelines. The investigation focused on analyzing the proportion of oral streptococcal IE across different risk groups in two distinct periods (1: 2000-2008; 2: 2009-2022). Logistic regression models, adjusted for various factors, were employed. Results: Of 752 IE cases, 163 occurred in period 1, and 589 in period 2. Oral streptococci caused 22% of cases. Proportions of streptococcal IE in period 1 versus period 2 were 24% versus 16% in high-risk, 24% versus 39% in moderate-risk, 33% versus 7% in low-/unknown-risk, and 18% versus 14% in no-risk patients. Compared to the other risk groups, the moderate-risk group had a 22% higher chance of oral streptococcal IE in period 2. After multivariable adjustment, moderate-risk patients had twice the risk of oral streptococcal IE compared to period 1 (OR: 2.59 [95% CI: 1.16-5.81]). Among moderate-risk conditions, congenital valve anomalies were associated with oral streptococcal IE (unadjusted OR: 2.52 [95% CI: 1.71-3.71]). Conclusions: Oral streptococcal IEs increased in the moderate-risk group of patients after the AP guideline change. Exploring the potential necessity for expanding AP indications to certain patient groups with congenital valve anomalies may be warranted.