Materiały źródłowe

• #1 Bicuspid Aortic Valve: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/893523-overview

  Bicuspid aortic valves may be present in as many as 1-2% of the population. Because the bicuspid valve may be entirely silent during infancy, childhood, and adolescence, these incidence figures may be underestimated and are not generally included in the overall incidence of congenital heart disease. […] A recent report suggests much lower than expected prevalence in African-Americans. […] The male-to-female ratio is 2:1 or greater. Sex is not a predictive variable in the natural history of bicuspid aortic valve. A recent prospective echocardiographic study in newborn infants showed a prevalence of bicuspid aortic valve in 7.1 per 1,000 male newborns versus 1.9 per 1,000 female newborns. […] Bicuspid aortic valve may be identified in patients of any age, from birth through the 11th decade of life. It may be only an incidental finding at autopsy. Bicuspid aortic valve may remain silent and be discovered as an incidental finding on echocardiographic examination of the heart.

• #1 Bicuspid Aortic Valve: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/893523-overview

  Critical aortic stenosis and infective endocarditis may be considered relatively early sources of morbidity for patients with bicuspid aortic valve. Critical aortic stenosis may occur in infancy and may be associated with a bicuspid valve. […] Stenosis of a bicuspid aortic valve is more likely to develop in persons older than 20 years and is caused by progressive sclerosis and calcification. High levels of serum cholesterol have been associated with more rapidly progressive sclerosis of the congenitally bicuspid aortic valve. […] Bicuspid aortic valve was identified in 167 (0.8%) of 20,946 young Italian military conscripts. Of these, 110 were found to have either mild or moderate aortic insufficiency.

• #2 Epidemiology of aortic valve stenosis (AS) and of aortic valve incompetence (AI): is the prevalence of AS/AI similar in different parts of the world?

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-18/epidemiology-of-aortic-valve-stenosis-as-and-of-aortic-valve-incompetence-ai

  There are large differences in the epidemiology of aortic valve disease between high-income and low-income countries. […] The prevalence of severe aortic stenosis in those aged 75 years is 3.4% in Europe and the USA. […] A bicuspid aortic valve (BAV) is the most common form of congenital heart disease, being found in approximately 0.5-0.8% of the population. […] Projections in high-income countries uniformly predict an increase in the burden of disease. […] Once RHD prevalence begins to decrease, CAVD can be expected to play a relatively more important role as a cause of AS in low-income countries. […] The high prevalence of risk factors for CAVD such as hypertension and increasing life expectancy, mean that there is likely to be a substantial future burden of CAVD in low-income countries. […] Epidemiologic studies have established equal rates of AS among men and women; however, important sex differences exist.

• #3 Bicuspid aortic valve

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-13/Bicuspid-aortic-valve

  Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality, affecting approximately 1%-2% of the general population. […] The prevalence of ascending aorta dilatation in patients with BAV ranged from 7.5% to 79%, according to the study of aortas in the population and region examined. […] Recent data suggest that BAV increases the risk of aortic dissection nine-fold over that in the general population. […] Coarctation of aorta is another major abnormality that can be associated with 20% or more of BAV. […] The mainstay of diagnosis is echocardiography (transthoracic or transoesophageal) which can provide a definitive diagnosis in the majority of patients with 92% sensitivity and 96% specificity when images are adequate. […] The recent ESC guidelines for valvular heart disease management recommend that surgery should be considered for any patient with aortic root diameter of 55 mm. […] In patients with BAV, surgery in aortic diameters of 50 mm should be based on patient age, body size, comorbidities, type of surgery, and the presence of additional risk factors.

• #4 Prevalence of Bicuspid Aortic Valve and Associated Aortopathy in Newborns in Copenhagen, Denmark

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7873775/

  Among newborns in Copenhagen, the prevalence of BAV was 0.77%. Aortopathy was common in newborns with BAV, suggesting that it also represents a fetal malformation. […] This population epidemiology study describes the prevalence of bicuspid aortic valve (BAV) and BAV subtypes, and of accompanying enlarged aortic diameter or coarctation, in Danish newborns born in Copenhagen 2016-2018. […] The prevalence of bicuspid aortic valve was 0.77%; of these, aortopathy was present in 33%. […] Aortopathy was seen in 65 newborns (33.2%) with BAV (62 with aortic z score 3; 3 with coarctation). […] The observed male predominance of BAV is in accordance with the common assumption of male predominance, although less pronounced than previously reported. […] This study provides an estimate of prevalence of BAV and BAV subtypes among newborns in Copenhagen. Aortopathy was common in newborns with BAV, suggesting that it also represents a fetal malformation.

• #5 A Comprehensive Review of Management Strategies for Bicuspid Aortic Valve (BAV): Exploring Epidemiology, Aetiology, Aortopathy, and Interventions in Light of Recent Guidelines

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10531880/

  BAV has a prevalence of 0.771.4% (potentially higher when asymptomatic patients are included) […] BAV represents the congenital heart disease (CHD) associated with the highest mortality rate. […] The prevalence of BAV among first-degree relatives of individuals with BAV stands at approximately 1015%. […] In light of this, both American and European guidelines released over the past decade advocate for screening through transoesophageal echocardiography (TEE) among primary family relatives, with varying levels of evidence supporting this recommendation falling within class IIa/b. […] The management of BAV presents a complex and critical challenge in cardiovascular medicine. […] The watch-and-wait strategy is generally recommended only for asymptomatic patients with preserved left ventricular ejection fraction (LVEF) and normal aortic diameters, although such cases represent a small proportion of BAV patients encountered in clinical practice. […] Therefore, it is crucial to provide comprehensive counselling to patients with BAV disease and to establish appropriate surgical plans for this patient population and their families.

• #6 A Comprehensive Review of Management Strategies for Bicuspid Aortic Valve (BAV): Exploring Epidemiology, Aetiology, Aortopathy, and Interventions in Light of Recent Guidelines

  https://www.mdpi.com/2308-3425/10/9/398

  BAV has a prevalence of 0.77–1.4% (potentially higher when asymptomatic patients are included) […] BAV represents the congenital heart disease (CHD) associated with the highest mortality rate. […] The prevalence of BAV among first-degree relatives of individuals with BAV stands at approximately 10–15%. […] In light of this, both American and European guidelines released over the past decade advocate for screening through transoesophageal echocardiography (TEE) among primary family relatives, with varying levels of evidence supporting this recommendation falling within class IIa/b.

• #7 Recognizing and Treating Bicuspid Aortic Valve Disease

  https://consultqd.clevelandclinic.org/recognizing-and-treating-bicuspid-aortic-valve-disease

  Bicuspid aortic valve (BAV) disease is one of the more common congenital heart conditions encountered in clinical practice. The estimated prevalence in the general population is 1-2%, with a predominance in males at a 4:1 ratio. […] Once a case of BAV disease is diagnosed, it is important to recognize that among families, as many as 10-15% of first-degree relatives may harbor BAV disease. Because of this prevalence, the screening of first-degree family members is recommended. Screening usually is done with a TTE to assess aortic valve anatomy and its function, as well as screen for dilatation of the ascending aorta.

• #8 Valvular Heart Disease Epidemiology

  https://www.mdpi.com/2076-3271/10/2/32

  Mortality rates have decreased in developed countries with the advent of new interventions, though no therapies are currently available to prevent the disease. […] While the bicuspid aortic valve is the most common form of congenital heart disease, occurring in 1–2% of the general population, it accounts for the presentation of 25% of patients >80 years who are referred for aortic valve replacement and 10% of patients who are undergoing TAVR. […] Bicuspid aortic valve is more prevalent among patients with Turner’s syndrome and other rare congenital diseases, with a prevalence of 6.4% among first-degree relatives with the condition, suggesting familial clustering. […] Early screening and surgical intervention has been proposed for bicuspid valve patients, though there is no current standard of care. […] Congenital bicuspid aortic valves are found in a lower frequency in African Americans. […] In Asian populations, a higher prevalence of bicuspid aortic valve (BAV) is seen in Chinese patients.

• #9 Bicuspid aortic valve – Wikipedia

  https://en.wikipedia.org/wiki/Bicuspid_aortic_valve

  Bicuspid aortic valves are the most common cardiac valvular anomaly, occurring in 12% of the general population. It is twice as common in males as in females. […] Bicuspid aortic valve is a heritable condition, with a demonstrated association with mutations in the NOTCH1 gene. Its heritability is as high as 89%. Both familial clustering and isolated valve defects have been documented. Recent studies suggest that BAV is an autosomal dominant condition with incomplete penetrance. Other congenital heart defects are associated with bicuspid aortic valve at various frequencies, including coarctation of the aorta. […] Bicuspid aortic valve abnormality is also the most observed cardiac defect in Turner syndrome.

• #10 Bicuspid aortic valve: Basics and beyond | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/85/10/779

  Close relatives should be evaluated for aortic valve and thoracic aortic disease. […] The American College of Cardiology (ACC) and the American Heart Association (AHA), backed by radiologic and cardiovascular associations, concur in recommending echocardiographic screening and routine screening of the thoracic aorta for aortic root dilation in first-degree relatives (ie, siblings, parents, and children) of patients with bicuspid aortic valve (class I recommendation).

• #10 Bicuspid aortic valve: Basics and beyond | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/85/10/779

  Bicuspid aortic valve, one of the most common congenital cardiac malformations, is associated with other valvular abnormalities and aortopathies. It increases the risk of thoracic aortic aneurysm and dissection, infective endocarditis, and sudden cardiac death. Patients require regular surveillance for progressive valvular disease and aortopathy. […] Regular surveillance with transthoracic echocardiography (TTE) is required. […] If initial imaging is normal and there is no aortic dilation, repeat imaging should be done every 5 to 10 years. If any abnormality is found, annual surveillance is needed. […] Because complications may be life-threatening, early detection of progressive disease by regular screening is critical. Echocardiographic evaluation of valvular function, ventricular dimensions and function, and diameter of the aortic root and ascending aorta should be performed in every patient with bicuspid aortic valve. If initial imaging is normal and there is no aortic dilation, imaging should be repeated every 5 to 10 years. If any abnormality is found, repeat imaging is needed every year.

• #11 Bicuspid aortic valve – British Heart Valve Society

  https://bhvs.org/heart-valve-conditions/bicuspid-aortic-valve/

  About 1 in 50 people are born with an aortic valve with two leaflets (a bicuspid aortic valve.) This is due to abnormal development of the valve whilst growing in the womb and is known as a congenital abnormality. The condition is more common in men than women. […] Most people with bicuspid aortic valve will not experience any symptoms and will be simply kept under surveillance. […] Most people with bicuspid aortic valve will not need any treatment and will just be kept under regular surveillance. […] There is a small risk (around 1 in 10 to 20) that your children and siblings may also have a bicuspid aortic valve (or related heart abnormalities). In some cases, a single one-off screening echocardiogram (echo or cardiac ultrasound) of your family members is recommended. If this is the case your doctor will discuss this with you at your clinic appointment.

• #12 Bicuspid aortic valve: General management in adults – UpToDate

  https://www.uptodate.com/contents/bicuspid-aortic-valve-general-management-in-adults

  Bicuspid aortic valve condition is a valvulo-aortopathy (affecting both the aortic valve and the thoracic aorta). Adults with bicuspid aortic valves commonly develop aortic valve dysfunction and disease of the ascending aorta and therefore require surveillance and appropriate management of these conditions. The management of bicuspid aortic valve disease in adults includes surveillance, timely intervention for valve disease and aortopathy, treatment of hypertension, measures to address the risk of infective endocarditis, counseling patients on physical activity, and management prior to and during pregnancy. […] General noninterventional management for adults with bicuspid aortic valve will be discussed here.

• #13 Variability in surveillance practice for patients with diagnosis of bicuspid aortic valve syndrome | Scientific Reports

  https://www.nature.com/articles/s41598-022-25571-x

  In patients with bicuspid aortic valves, guidelines call for regular follow-up to monitor disease progression and guide intervention. […] Follow-up care for patients with bicuspid aortic valve was highly variable, and surveillance imaging was sparse despite guidelines. […] The 2018 American Association for Thoracic Surgery (AATS) guidelines for the management of BAV recommend serial evaluations of the aorta by transthoracic echocardiogram (TTE) with intervals tailored to the presence and severity of aortic dilation. […] Current guidelines suggest that the frequency and type of surveillance should be based on severity of aortic dilatation. […] Overall, follow-up and use of surveillance imaging of the aorta or the aortic valve may be variable despite awareness of guideline recommendations. There is an urgent need for systematic surveillance and implementation of clinical follow-up mechanisms to monitor this patient population with increased risk of progressive valvulopathy and aortopathy.

• #14

  https://scholars.duke.edu/display/pub733354

  Our approach to the management of the patient with a bicuspid aortic valve (BAV) takes several factors into consideration. […] In general, we follow patients with a BAV and moderate valve dysfunction (AS or AR) with yearly surveillance transthoracic echocardiography for left ventricular function, jet velocity, gradient, and valve area with AS, whereas left ventricular (LV) function and LV dimensions are monitored for patients with AR. […] All patients with a bicuspid valve should have evaluation of the aorta with a MRI or CT angiography at some point, as 50% of BAV patients have aortic root involvement. […] For patients with mild aortic dilation, surveillance aortic imaging is usually performed every 3-5 years. However, for those with greater degrees of aortic dilation (aortic diameters 4.0 cm) or notable interval change in dimensions, then aortic imaging every year is conducted.

• #15 Bicuspid aortic valve | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/bicuspid-aortic-valve?lang=us

  Bicuspid aortic valve (BAV) refers to a spectrum of deformed aortic valves with two functional leaflets or cusps which are often unequal in size. […] The estimated incidence of a congenital bicuspid valve in the general population is thought to be ~2%. They may be more common in males. […] A congenitial biscuspid aortic valve is considered to be one of the most common causes of isolated aortic stenosis. It is considered a major cause of aortic valve disease in young adults. […] Transthoracic echocardiography is typically an adequate imaging modality for identifying the presence of a bicuspid aortic valve, with sensitivity and specificity approaching 92% and 96% respectively given the presence of adequate sonographic windows. It is also used to surveil the development of valvular dysfunction and the associated aortopathy.

• #15 Bicuspid aortic valve | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/bicuspid-aortic-valve?lang=us

  At the time of writing, CT has a much higher reported detection rate for bicuspid valves. […] Cardiac MRI has the advantage of demonstrating the dynamic motion of the bicuspid valve when heavily calcified valves make echocardiography difficult to interpret. Furthermore, MRI can provide non-invasive assessment of the ascending aortic diameter and the presence of coarctation in a single study.

• #16 Speaking a common language: the international consensus on bicuspid aortic valve nomenclature and classification – Michelena- Annals of Cardiothoracic Surgery

  https://www.annalscts.com/article/view/16952/html

  Advanced imaging modalities are also critical in assessing the BAV condition: electrocardiographic (ECG)-gated cardiac CT and ECG-gated MR. […] The definition of aortic aneurysm is rarely applied in clinical practice, and the term aneurysm carries a somber or dismal connotation for patients. […] The importance of recognizing BAV aortopathic phenotypes is that their presence and association with specific valvular phenotypes and dysfunction patterns may imply different clinical histories for a patient with BAV. […] The root phenotype has been associated with greater rates of acute aortic dissection in the postoperative follow-up of patients with BAV who had undergone simple aortic valve replacement compared to the ascending phenotype.

• #17 Bicuspid Aortic Valves: Outlook Good, but Surveillance Necessary

  https://www.medscape.org/viewarticle/580659

  The largest study to date to follow a population of young adults with bicuspid aortic valves over nine years has found that their survival is similar to the general population of the same age. […] There was a high frequency of cardiac events among those with the congenital condition, however, necessitating intervention. […] This work gives an important message that yes, the outlook is good for these patients, but the fact is they also need regular surveillance by somebody who is familiar with this condition. […] The researchers identified several factors that were independently associated with primary cardiac events: increasing age, severity of aortic stenosis, and severity of aortic regurgitation. […] The good news is that the overall mortality rate was not significantly different from population estimates. The concerning news is that, with time, a significant number of these patients have events requiring intervention, usually surgery to replace the aortic valve because of a leaky or narrowing valve or due to enlargement of the aorta.

• #18 Heart valve disease module 1: epidemiology – The British Journal of Cardiology

  https://bjcardio.co.uk/2016/03/heart-valve-disease-module-1-epidemiology-2/4/

  The most common congenital anomaly is bicuspid aortic valve and this is found in 0.50.8% of large population studies although it is reported in up to two thirds all valves excised during valve replacement for aortic stenosis and up to 2.0% of the population based on autopsy series. […] The risk of a bicuspid valve or aortopathy is about 10% in first-degree relatives of probands. The ratio of male to female is approximately 2:1. […] During a 20 year follow-up, 24% of patients with a bicuspid aortic valve developed severe stenosis or regurgitation requiring surgery. Events are far more common in those with even mild valve thickening at baseline, with surgical rates of 75% at 12 years in the presence of thickening compared with only 8% without thickening. […] Bicuspid aortic valve should be regarded as a general thoracic aortopathy and is associated with significant dilatation of the aorta (40 mm) in about 20% of cases. Approximately one half affects the root, and the rest the ascending aorta.

• #19 Bicuspid Aortic Valve and Bicuspid Aortopathy Nomenclature and Classification

  https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2021/09/02/18/37/Summary-International-Consensus-Statement

  The congenital bicuspid aortic valve (BAV) condition is a valvulo-aortopathy with substantial heterogeneity in its clinical expressions, associated disorders, complications, and prognosis. […] Typical valvulo-aortopathy is characterized by progressive valvular dysfunction and/or aorta dilation without other major associated disorders. This presentation commonly is diagnosed in young adult and adult populations, requires long-term surveillance, and commonly requires invasive treatment. […] Common complications of typical valvulo-aortopathy, in order of frequency, are aortic stenosis (AS) requiring intervention, ascending thoracic aorta dilation that may require repair, aortic regurgitation (AR) requiring intervention, mitral valve prolapse requiring intervention, infective endocarditis, and aortic dissection.

• #19 Bicuspid Aortic Valve and Bicuspid Aortopathy Nomenclature and Classification

  https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2021/09/02/18/37/Summary-International-Consensus-Statement

  The authors propose reference to aortic dilation rather than aortic aneurysm. […] There are two major forms of BAV aortic dilation phenotypes. The ascending phenotype, with dilation preferentially in the tubular ascending aorta accounts for approximately 70% of BAV aortopathy cases. The root phenotype, with dilation preferentially within the aortic root, accounts for approximately 20% of BAV aortopathy cases. […] In some cases, aortic dilation is not dominant at a single segment, and in some cases, localized aorta dilation can evolve during follow-up.

• #20 Bicuspid aortic stenosis epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Bicuspid_aortic_stenosis_epidemiology_and_demographics

  Bicuspid aortic stenosis is the most common congenital abnormality of the heart accounting for 5% of congenital heart defects and it occurs in 1-2% of the population. […] The incidence of bicuspid aortic valve can be as high as 10% to 20% in families affected with the valve problem. […] Aortic stenosis occurs in this condition usually in patients in their 40s or 50s, an average of 10 years earlier than can occur in people with congenitally normal aortic valves. […] Bicuspid aortic stenosis is more common in men than women (3:1 to 5:1).

• #21 Bicuspid Aortic Valve: Current Therapeutic Strategies | IntechOpen

  https://www.intechopen.com/chapters/88437

  Surveillance for BAV syndrome patients is performed based on the pathophysiology of the aortic valve phenotype or aortic phenotype. […] Current guidelines suggest family screening with echocardiography for all first-degree relatives with BAV probands. […] Lifetime follow-up and aortic surveillance is also important as the timing of incidence of valve or aortic disease may be different amongst different family members.

• #22 Managing Bicuspid Aortic Valve Disease

  https://consultqd.clevelandclinic.org/bicuspid-aortic-valve-disease-multifaceted-challenges-demand-versatile-management

  A BAV occurs in about 1% to 2% of the population and may be associated with an inherited connective tissue disorder. […] Many of these patients develop valvular regurgitation that requires intervention. […] In the Cleveland Clinic experience, more than one-third develop aortic enlargement. […] A study comparing Cleveland Clinic patients who underwent aortic root replacement with either BAV reimplantation (n = 92) or tricuspid aortic valve reimplantation (n = 515) from 2002 to 2017 found similar short-term and five-year outcomes among 71 matched pairs. […] However, higher gradients, less ventricular reverse remodeling and more aortic valve reoperations in the BAV group indicated a need for continued surveillance in such patients. […] Another Cleveland Clinic study analyzed more than two decades of data (1998-2020) from patients who underwent BAV repair with (n = 419) or without (n = 421) ascending aorta replacement.

• #22 Managing Bicuspid Aortic Valve Disease

  https://consultqd.clevelandclinic.org/bicuspid-aortic-valve-disease-multifaceted-challenges-demand-versatile-management

  At 10 years, across the overall cohort, survival was 95% after BAV repair with aorta replacement and 96% after BAV repair alone, and rates of freedom from reoperation were 79% and 75%, respectively. […] Among the matched pairs, severe aortic regurgitation developed within 10 years in 9.1% of patients undergoing BAV repair alone and 11% of those with BAV repair plus aorta replacement. […] Dr. Lou notes that an important focus of the study was to determine the importance of replacing the ascending aorta with a tube graft for the sole purpose of stabilizing the sinutubular junction (STJ). […] However, comparison of the matched pairs revealed that stabilization of the STJ with ascending aorta replacement at the time of BAV repair had minimal effect on long-term repair durability.

• #23 Implications of Bicuspid Aortic Valve Disease and Aortic Stenosis/Insufficiency as Risk Factors for Thoracic Aortic Aneurysm

  https://www.imrpress.com/journal/RCM/24/6/10.31083/j.rcm2406178/htm

  This review aims to summarize the latest scientific knowledge on the link between AV disease (AI, AS, BAV) and aortopathies of the proximal aorta (root/ascending), as well as identifying current gaps in the management of TAA patients with AV disease. […] Current criteria for concomitant aortic replacement when undergoing surgery for AV dysfunction is 4.5 cm, irrespective of AV anatomy or dysfunction type, holding a Class 2a recommendation for both TAV and BAV. […] Comprehensive aortic surveillance programs should not only include longitudinal anatomic analysis of the aortic root and ascending aorta via computed tomography (CT)/MRI scan, but also morphologic and functional analysis of the AV by echocardiography.

• #23 Implications of Bicuspid Aortic Valve Disease and Aortic Stenosis/Insufficiency as Risk Factors for Thoracic Aortic Aneurysm

  https://www.imrpress.com/journal/RCM/24/6/10.31083/j.rcm2406178/htm

  Bicuspid Aortic Valves (BAV) are associated with an increased incidence of thoracic aortic aneurysms (TAA). […] Patients with AV disease and concomitant TAA lack a widely accepted diagnostic approach, one that integrates our knowledge on aortic valve pathophysiology and encompasses multi-modality imaging approaches. […] Given the asymptomatic nature of TAA, serial surveillance after diagnosis using various imaging techniques like echocardiography, computed tomography (CT), and magnetic resonance imaging (MRI) is crucial. […] Currently, there is no comprehensive approach in managing patients with AV disease and TAA that incorporates all imaging techniques and necessary knowledge concerning AV disease-TAA pathophysiology; an approach essential to providing accurate disease prognosis and appropriate monitoring in these patients.

• #24 NHS England » Guide to implementing patient initiated follow-up and regular surveillance imaging for patients with mild to moderate heart valve disease

  https://www.england.nhs.uk/long-read/guide-to-implementing-pifu-imaging-mild-to-moderate-heart-valve-disease/

  Patients with the following conditions: […] bicuspid aortic valve with non-severe valve disease […] Patients with bicuspid aortic valve or co-existent untreated valve lesions may not be suitable for PIFU. […] Condition: Normally functioning or mildly diseased bicuspid aortic valve […] Imaging follow-up interval: 2-5 years (12 months in the presence of aortic dilatation 40mm) […] Comments: Note association of aortopathy with this condition, although aortic dissection remains rare. […] If echocardiogram is unable to assess for aortopathy alone, patient is not suitable for PIFU pathway.

• #25 Bicuspid Aortic Valve (BAV) Treatment | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/bicuspid-aortic-valve-program/

  The Heart Center at Lurie Childrens is dedicated to monitoring and caring for more than 1,000 infants, children and teenagers with bicuspid aortic valve (BAV) disease. BAV is the most common form of congenital heart disease, affecting between 1-2% of the general population. […] Bicuspid aortic valve may be an inherited condition, occurring in up to 9% of first-degree relatives. Recent studies have shown that first-degree relatives may also have aortic enlargement in the absence of a BAV. Screening is recommended for all first-degree relatives (such as parents and siblings) of a person diagnosed with BAV. Early diagnosis can lead to the proper surveillance, management and improved long-term outcomes. […] The Cardiovascular Bridge Program also manages a centralized database that collects clinical information regarding BAV over time. This information will allow us to learn more about the natural history, physiologic and genetic mechanisms of this disease so that we can improve long-term outcomes.

• #26 Changing epidemiology of calcific aortic valve disease: 30-year trends of incidence, prevalence, and deaths across 204 countries and territories | Aging

  https://www.aging-us.com/article/202942/text

  Calcific aortic valve disease (CAVD) is associated with increased morbidity and mortality. We aimed to elucidate the 30-year epidemiology of CAVD globally. Global CAVD incidence, prevalence, and deaths increased 3.51-, 4.43-, and 1.38-fold from 1990 to 2019, respectively, without any decreasing trends, even after age standardization. In 2019, Slovenia had the highest age-standardized rate (ASR) of CAVD incidence (62.21/100,000 persons) and prevalence (1,080.06/100,000) whereas Cyprus had the highest ASR of deaths (8.20/100,000). Population aging was an important contributor to incidence. Compared with women, more men had CAVD and men had earlier peaks in disease prevalence. High systolic blood pressure, diet high in sodium, and lead exposure were the main risk factors for deaths owing to CAVD. The estimated annual percentage change, a measure to estimate the variation of ASR, was significantly associated with the ASR and sociodemographic index (SDI) in 2019 for incidence and prevalence across all 204 countries and territories (all p<0.0001). With increased lifespan and risk factors, the overall burden of CAVD is high and remains on the rise, with differences by sex, age, and SDI level. Our findings serve to sound the alarm for organizations, institutions, and resources whose primary purpose is to improve human health.

• #26 Changing epidemiology of calcific aortic valve disease: 30-year trends of incidence, prevalence, and deaths across 204 countries and territories | Aging

  https://www.aging-us.com/article/202942/text

  The incidence of CAVD varied significantly from nation to nation. In 2019, the highest number of incident cases (117,080) was recorded in the United States, followed by Japan (71,837), China (54,965), and the Russian Federation (54,152). Moreover, Slovenia, Hungary, and Romania had the highest ASIR, with 62.21/100,000 persons, 56.24/100,000 persons, and 54.92/100,000 persons, respectively. […] The most CAVD deaths in 2019 were recorded in the United States, followed by Germany and Japan (248,256, 13,154, and 12,868, respectively). Cyprus, Slovenia, and Norway presented the largest ASDR with 8.20/100,000 persons, 6.77/100,000 persons, and 5.72/100,000 persons, respectively. […] Since 1990, no SDI or GBD regions have shown a decreasing trend in CAVD deaths or ASDR. In 2019, among the five SDI quintiles, the highest number of deaths (80,211) and highest ASDR (3.35/100,000 persons) were both identified in high SDI quintiles.

• #26 Changing epidemiology of calcific aortic valve disease: 30-year trends of incidence, prevalence, and deaths across 204 countries and territories | Aging

  https://www.aging-us.com/article/202942/text

  Globally, cases of CAVD have increased continuously over the past 30 years, with no downward trends observed in the ASIR, ASPR, or ASDR. Population aging was an important contributor to incident cases. Moreover, differences were present in the sex and age distributions of prevalent cases. A high SBP, a diet high in sodium, and exposure to lead were the main risk factors of CAVD deaths. Moreover, the EAPC was significantly related to the ASR and SDI in 2019 for incidence and prevalence. Generally, the findings of the present study will be helpful in elucidating the global disease burden of CAVD, to develop more effective and efficient CAVD prevention and treatment strategies.

• #27 Evaluation of TAVR Compared to Surveillance for Patients With Asymptomatic Severe Aortic Stenosis – EARLY TAVR

  https://www.acc.org/latest-in-cardiology/clinical-trials/2024/10/24/14/42/early-tavr

  The EARLY TAVR trial showed that TAVR for stage C aortic stenosis was beneficial compared with clinical surveillance. […] Patients with asymptomatic severe aortic stenosis (stage C aortic stenosis) were randomized to TAVR (n = 455) vs. clinical surveillance (n = 446). […] Bicuspid aortic valve: 8.1%. […] In the clinical surveillance group, 87% underwent aortic valve replacement during follow-up. […] Among patients with asymptomatic severe aortic stenosis (stage C aortic stenosis), early TAVR was beneficial. Early TAVR compared with clinical surveillance resulted in a lower incidence of the composite outcome of death, stroke, or unplanned hospitalization for cardiovascular cause. This trial will be used to inform future guideline recommendations regarding the management of stage C aortic stenosis.

• #28 Clinical Outcomes in Patients With Bicuspid Aortic Valves and Ascending Aorta >50 mm Under Surveillance – AortaHub

  https://www.aortahub.com/en/publicacionesrecient/clinical-outcomes-in-patients-with-bicuspid-aortic-valves-and-ascending-aorta-50-mm-under-surveillance/

  Of 875 consecutive BAV patients (age 60 13 years, 86% men, aortic diameter 51 mm [interquartile range (IQR): 50-53 mm]), 328 (37%) underwent early surgery 3 months from index TTE. […] Of the remaining 547 patients under surveillance, 496 had diameters 50 to 54 mm and 51 had diameters 55 mm and were collectively followed for 7.51 (IQR: 3.98-12.20) years. […] AoD occurred in 9/496 (1.8%) patients for an incidence of 0.4 cases per 100 person-years, surgical mortality was 5/266 (1.9%); and moderate aortic stenosis (but not aorta size) was associated with all-cause death, hazard ratio: 2.05 (95% Cl: 1.32-3.20), P = 0.001. […] Conversely, in 547 total patients under surveillance (including 50-54 mm and 55 mm), both aorta size and moderate aortic stenosis were associated with all-cause death (both 0.027).

• #28 Clinical Outcomes in Patients With Bicuspid Aortic Valves and Ascending Aorta >50 mm Under Surveillance – AortaHub

  https://www.aortahub.com/en/publicacionesrecient/clinical-outcomes-in-patients-with-bicuspid-aortic-valves-and-ascending-aorta-50-mm-under-surveillance/

  In BAV patients with ascending aorta 50 to 54 mm under surveillance, AoD incidence is low and the overall rates of AoD and surgical mortality are similar, suggesting clinical equivalence between surgical and surveillance strategies. […] Conversely, patients with aortas 55 mm should undergo surgery.

• #29 Moderate aortic stenosis: culprit or bystander? | Open Heart

  https://openheart.bmj.com/content/9/1/e001743

  Emerging data have revealed that the prognosis of individuals with moderate AS is unfavourable, in some cases approaching that of severe AS, with unchanged event-free survival over the last few decades. […] Large observational studies have demonstrated a high overall mortality associated with moderate AS. […] Patients with both moderate AS and reduced LVEF have high rates of cardiovascular morbidity and mortality. […] Several factors can influence the unfavourable prognosis of moderate AS demonstrated in recent studies, including the misclassification of severe AS as moderate AS, the associated LV hypertrophy, the existence of cardiovascular risk factors which colocalize with moderate AS, and the presence of rapid AS progressors. […] Randomised control trials are ongoing to determine whether patients with moderate AS benefit from earlier valvular intervention than the current guideline-recommended approach.

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