Streptococcus alactolyticus is a non-motile Gram-positive, catalase-negative cocci, a part of group D Streptococci. In the literature, S. alactolyticus is documented as a causative agent of infective endocarditis, demonstrated by blood cultures in only four other cases, representing an extremely rare circumstance. Here, we describe a case of infective endocarditis due to S. alactolyticus in a young patient known with a bicuspid aortic valve and associated with a sigmoid precancerous polyp. The patient was also known to have blood hypertension and type II diabetes. Symptoms at the debut appeared insidiously and were non-specific: fatigue, loss of appetite, weight loss, night sweats, and fever. They lasted for the entire period of the illness with transient improvement during the courses of antibiotics. He followed more antibiotic courses prescribed for various clinical diagnoses. Each round of antibiotic treatment transitorily alleviated the symptoms, which reappeared each time after the cessation. The correct diagnosis was made only about three months after the appearance of the first clinical manifestations. This was based on ultrasound criteria (presence of vegetation and lesions of aortic cusps) and microbiological criteria (isolation of S. alactolyticus in blood cultures). A course of six weeks of ceftriaxone was considered the opportune antibiotic therapy. Similar to all other cases described in the literature, our patient presented important damage to the valvular tissue and required cardiac surgery to re-establish the normal function of the valve. The surgery consisted of the excision of the severely affected natural aortic valve and her replacement with a mechanical prosthetic valve. Following medical and surgical treatment, the patient is completely healed and has a normal life. Our case is noteworthy because of the scarcity of the involvement of S. alactolyticus in the pathogeny of infective endocarditis. This is the fifth published case with this etiology, and an overview of all five cases is provided in the article.

UNASSIGNED: Neural crest cells (NCCs) are multipotent and are attributed to the combination of complex multimodal gene regulatory mechanisms. Cardiac neural crest (CNC) cells, originating from the dorsal neural tube, are pivotal architects of the cardio-neuro-vascular domain, which orchestrates the embryogenesis of critical cardiac and vascular structures. Remarkably, while the scientific community compiled a comprehensive inventory of neural crest derivatives by the early 1980s, our understanding of the CNC\'s role in various cardiovascular disease processes still needs to be explored. This review delves into the differentiation of NCC, specifically the CNC cells, and explores the diverse facets of non-syndromic cardiovascular neurocristopathies.
UNASSIGNED: A systematic review was conducted as per the PRISMA Statement. Three prominent databases, PubMed, Scopus, and Embase, were searched, which yielded 1,840 studies. We excluded 1,796 studies, and the final selection of 44 studies formed the basis of this comprehensive review.
UNASSIGNED: Neurocristopathies are a group of genetic disorders that affect the development of cells derived from the NC. Cardiovascular neurocristopathy, i.e., cardiopathy and vasculopathy, associated with the NCC could occur in the form of (1) cardiac septation disorders, mainly the aortico-pulmonary septum; (2) great vessels and vascular disorders; (3) myocardial dysfunction; and (4) a combination of all three phenotypes. This could result from abnormalities in NCC migration, differentiation, or proliferation leading to structural abnormalities and are attributed to genetic, familial, sporadic or acquired causes.
UNASSIGNED: Phenotypic characteristics of cardiovascular neurocristopathies, such as bicuspid aortic valve and thoracic aortic aneurysm, share a common embryonic origin and are surprisingly prevalent in the general population, necessitating further research to identify the underlying pathogenic and genetic factors responsible for these cardiac anomalies. Such discoveries are essential for enhancing diagnostic screening and refining therapeutic interventions, ultimately improving the lives of individuals affected by these conditions.

Aortic stenosis is one of the most prevalent cardiac valvular diseases throughout the world and has a significant impact on quality of life. While there are several etiologies, we will be discussing the case of a male in his mid-thirties of southeast Asian descent with a bicuspid aortic valve which was found to be functionally unicuspid and complicated by aortic dilation. Following a comprehensive review of literature, it appears this subset of aortic stenosis is not commonly encountered. In addition to presenting this fascinating case, we will review the epidemiology, classification and management of aortic stenosis. Furthermore, we will examine the latest evidence-based literature on bicuspid aortic valve and unicuspid aortic valve and discuss interventions and diagnostic tools that may improve clinical prognosis.

Bicuspid aortic valve (BAV) is the most commonly encountered congenital malformation in the pediatric population, associated with aortic leaflet degeneration and aortopathy. However, studies on BAV and its complications in children are limited. We present the case of a 16-year-old with type 1B BAV with a raphe with fusion between the right and non-coronary cusps who exhibited severe aortic stenosis, regurgitation, and progressive dilatation of the ascending aorta. Surgical intervention, including aortic valve and aortic root replacement, was performed due to the patient\'s deteriorating condition. Histopathological examination revealed degenerative changes and calcifications in the aortic valve and mucoid fibrosis in the ascending aorta. The results are consistent with BAV patients being predisposed to aortic stenosis and regurgitation due to increased mechanical stress and hemodynamic abnormalities. Although more common in adults and a rare complication in pediatric patients, calcification was previously observed concurrently with rapid valve degeneration in our daily practice. Further studies are needed to improve our understanding of the mechanisms underlying BAV-related complications and refine treatment strategies for pediatric patients.

OBJECTIVE: bicuspid aortic valve (BAV) stands as the most prevalent congenital heart condition intricately linked to aortic pathologies encompassing aortic regurgitation (AR), aortic stenosis, aortic root dilation, and aortic dissection. The aetiology of BAV is notably intricate, involving a spectrum of genes and polymorphisms. Moreover, BAV lays the groundwork for an array of structural heart and aortic disorders, presenting varying degrees of severity. Establishing a tailored clinical approach amid this diverse range of BAV-related conditions is of utmost significance. In this comprehensive review, we delve into the epidemiology, aetiology, associated ailments, and clinical management of BAV, encompassing imaging to aortic surgery. Our exploration is guided by the perspectives of the aortic team, spanning six distinct guidelines.
METHODS: We conducted an exhaustive search across databases like PubMed, Ovid, Scopus, and Embase to extract relevant studies. Our review incorporates 84 references and integrates insights from six different guidelines to create a comprehensive clinical management section.
RESULTS: BAV presents complexities in its aetiology, with specific polymorphisms and gene disorders observed in groups with elevated BAV prevalence, contributing to increased susceptibility to other cardiovascular conditions. The altered hemodynamics inherent to BAV instigate adverse remodelling of the aorta and heart, thus fostering the development of epigenetically linked aortic and heart diseases. Employing TTE screening for first-degree relatives of BAV patients might be beneficial for disease tracking and enhancing clinical outcomes. While SAVR is the primary recommendation for indicated AVR in BAV, TAVR might be an option for certain patients endorsed by adept aortic teams. In addition, proficient teams can perform aortic valve repair for AR cases. Aortic surgery necessitates personalized evaluation, accounting for genetic makeup and risk factors. While the standard aortic replacement threshold stands at 55 mm, it may be tailored to 50 mm or even 45 mm based on patient-specific considerations.
CONCLUSIONS: This review reiterates the significance of considering the multifactorial nature of BAV as well as the need for further research to be carried out in the field.

Bicuspid aortic valve (BAV) is present in approximately 0.5%-2% of the general population, causing significant aortic stenosis (AS) in 12%-37% of affected individuals. Transcatheter aortic valve replacement (TAVR) is being considered the treatment of choice in patients with symptomatic AS across all risk spectra.
Aim Our study aims to compare TAVR outcomes in patients with BAV versus tricuspid aortic valves (TAV).
A comprehensive literature search was performed in PubMed, Web of Science, and Cochrane trials. Studies were included if they included BAV and TAV patients undergoing TAVR with quantitative data available for at least one of our predefined outcomes. Meta-analysis was performed by the random-effects model using Stata software.
Fifty studies of 203,288 patients were included. BAV patients had increased 30-day all-cause mortality (odds ratio [OR] = 1.23 [1.00-1.50], p = 0.05), in-hospital stroke (OR = 1.39 [1.01-1.93], p = 0.05), in-hospital and 30-day PPI (OR = 1.13 [1.00-1.27], p = 0.04; OR = 1.16 [1.04-1.13], p = 0.01) and in-hospital, 30-day and 1-year aortic regurgitation (AR) (OR = 1.48 [1.19-1.83], p < 0.01; OR = 1.79 [1.26-2.52], p < 0.01; OR = 1.64 [1.03-2.60], p = 0.04). Subgroup analysis on new-generation valves showed a reduced 1-year all-cause mortality (OR = 0.86 [CI = 0.75-0.98], p = 0.03), despite higher in-hospital and 30-day PPI (OR = 0.1.21 [1.04-1.41], p = 0.01; OR = 1.17 [1.05-1.31], p = 0.01) and in-hospital AR (OR = 1.62 [1.14-2.31], p = 0.01) in the BAV group. The quality of included studies was moderate-to-high, and only three analyses presented high heterogeneity.
TAVR is associated with comparable outcomes in patients with BAV and TAV. Careful selection of BAV cases by preprocedural assessment of valve anatomy and burden of calcification, pre- and post-procedural dilation, and implementing newer generations of valves may improve the safety and efficacy of TAVR in BAV patients.

UNASSIGNED: Antibiotic prophylaxis in bicuspid aortic valve patients is currently a matter of debate. Although it is no longer recommended by international guidelines, some studies indicate a high risk of infective endocarditis. We aim to evaluate the risk of native valve infective endocarditis in bicuspid aortic valve patients and compare to individuals with tricuspid aortic valve.
UNASSIGNED: Study search of longitudinal studies regarding infective endocarditis incidence in bicuspid aortic valve patients (compared with tricuspid aortic valve/overall population) was conducted through OVID in the following electronic databases: MEDLINE, CENTRAL, EMBASE; from inception until October 2020. The outcomes of interest were the incidence rate and relative risk of infective endocarditis. The relative risk and incidence rate (number of cases for each 10 000 persons-year) with their 95 % confidence intervals (95 %CI) were estimated using a random effects model meta-analysis. The study protocol was registered at PROSPERO CRD42020218639.
UNASSIGNED: Eight cohort studies were selected, with a total of 5351 bicuspid aortic valve patients. During follow up, 184 bicuspid aortic valve patients presented infective endocarditis, with an incidence rate of 48.13 per 10,000 patients-year (95 %CI 22.24-74.02), and a 12-fold (RR: 12.03, 95 %CI 5.45-26.54) increased risk compared with general population, after adjusted estimates.
UNASSIGNED: This systematic review and meta-analysis suggests that bicuspid aortic valve patients have a significant high risk of native valve infective endocarditis. Large prospective high-quality studies are required to estimate more accurately the incidence of infective endocarditis, the relative risk and the potential benefit of antibiotic prophylaxis.

Bicuspid aortic valve is the most common congenital heart disease and exposes patients to an increased risk of aortic dilation and dissection. Aortic dilation is a slow, silent process, leading to a greater risk of aortic dissection. The prevention of adverse events together with optimization of the frequency of the required lifelong imaging surveillance are important for both clinicians and patients and motivated extensive research to shed light on the physiopathologic processes involved in bicuspid aortic valve aortopathy. Two main research hypotheses have been consolidated in the last decade: one supports a genetic basis for the increased prevalence of dilation, in particular for the aortic root, and the second supports the damaging impact on the aortic wall of altered flow dynamics associated with these structurally abnormal valves, particularly significant in the ascending aorta. Current opinion tends to rule out mutually excluding causative mechanisms, recognizing both as important and potentially clinically relevant.

BACKGROUND: Valve-sparing aortic root replacement (VSARR) is a safe and effective surgical procedure to treat aortic root aneurysm. This meta-analysis aimed to investigate how this procedure might differ in patients with bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV).
METHODS: Meta-analysis with meta-regression and systematic review.
METHODS: Systematic search in the following databases: PubMed, Cochrane Central Register of Controlled Trials, and Embase.
METHODS: All observational studies of VSARR in patients with BAV or TAV were included in our study. Studies were included without any restrictions on language or publication date. A trial sequential analysis and a post-hoc meta-regression was performed on the main outcomes.
RESULTS: Eleven articles met the inclusion criteria. A total of 1138 patients in BAV group, and 2125 patients in TAV group. No significant differences in gender and age were observed between BAV and TAV patients. BAV and TAV patients showed no differences in in-hospital mortality rate [0.00% vs. 1.93%; RR (95% CI) 0.33 (0.09, 1.26), I2 = 0%, P = 0.11] and the rate of in-hospital reoperation [5.64% vs. 5.99%; RR (95% CI) 1.01(0.59, 1.73), I2 = 33%, P = 0.98]. The overall long-term mortality rate of BAV patients was better than that of TAV patients [1.63% vs. 8.15%; RR (95% CI) 0.34 (0.13, 0.86), I2 = 0%, P = 0.02]. During the follow-up observation period, patients in TAV group showed small but no statistic advantage in 3-year, 5-year, and over 10-year incidences of reintervention. Regarding the secondary endpoints, the two groups showed similar aortic cross-clamping time and total cardiopulmonary bypass time.
CONCLUSIONS: The VSARR techniques yielded similar clinical outcomes in both BAV and TAV patients. Although patients with BAV might have a higher incidence of reinterventions after initial VSARR, it is still a safe and effective approach to treat aortic root dilation with or without aortic valve insufficiency. TAV patients showed small but no statistic advantage in long-term (over 10 years) reintervention rate, which means, patients with BAV may face a higher risk of reintervention in the clinic.

UNASSIGNED: Bicuspid aortic valves have been excluded from randomized trials comparing transcatheter aortic valve replacement with surgical aortic valve replacement. We aimed to evaluate the outcomes of transcatheter aortic valve replacement versus surgical aortic valve replacement in patients with severe bicuspid aortic valve stenosis using a meta-analysis.
UNASSIGNED: MEDLINE and EMBASE were searched through March 2022 to identify observational studies comparing transcatheter aortic valve replacement and surgical aortic valve replacement for severe bicuspid aortic valve stenosis. Outcomes of interest were in-hospital outcomes, including all-cause mortality, stroke, vascular complication, permanent pacemaker implantation, acute kidney injury, blood transfusion, paravalvular leak, and all-cause mortality during follow-up.
UNASSIGNED: Four propensity score-matched studies and 54,047 patients (transcatheter aortic valve replacement, n = 3841; surgical aortic valve replacement, n = 50,206) yielding 3142 pairs using propensity score were included. Median follow-up periods were 21 to 24 months. There were no significant differences in in-hospital mortality (risk ratio, 0.69; 95% confidence interval, 0.40-1.20; P = .19) or stroke (risk ratio, 0.86; 95% confidence interval, 0.64-1.14; P = .29). Although transcatheter aortic valve replacement was associated with higher risks of permanent pacemaker implantation rate (risk ratio, 1.87; 95% confidence interval, 1.23-2.84; P = .0003), transcatheter aortic valve replacement was associated with lower risks of acute kidney injury (risk ratio, 0.58; 95% confidence interval, 0.38-0.88; P = .01) and transfusion (risk ratio, 0.25; 95% confidence interval, 0.21-0.29; P = .0001). There were no significant differences in in-hospital vascular complication, paravalvular leak, and all-cause mortality during follow-up.
UNASSIGNED: In selected patients with severe bicuspid aortic valve stenosis, no significant differences in in-hospital mortality or stroke were observed between transcatheter aortic valve replacement and surgical aortic valve replacement. Further investigations with long-term follow-up and morphological features are warranted.