Bicuspid aortic valve and its ascending aortopathy

Article Cardiac & Cardiovascular Systems

The Genetic Determinants of Aortic Distention

James P. Pirruccello et al.

Summary: Genetic determinants of aortic function influence the risk for stroke and coronary artery disease, and may lead to novel targets for medical intervention.

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY (2023)

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Article Cardiac & Cardiovascular Systems

Clinical Research Regional Vascular Changes and Aortic Dilatation in Pediatric Patients With Bicuspid Aortic Valve

Rawan K. Rumman et al.

Summary: Children with BAV have differences in vascular function that are confined to their proximal aorta, even in normal functioning BAV. The observed differences in vascular function are likely multi-factorial, with contributions from abnormal regional flow and a potential localized primary aortopathy.

CANADIAN JOURNAL OF CARDIOLOGY (2022)

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Article Cardiac & Cardiovascular Systems

Circulating microRNAs in the prediction of BAV aortopathy: do the expression patterns correlate between blood and aortic tissue?

Shiho Naito et al.

Summary: The study demonstrates a significant correlation between peripheral whole blood and aortic tissue miRs, suggesting that circulating miRs may reflect remodeling processes in the proximal aorta in patients with bicuspid aortopathy.

REVIEWS IN CARDIOVASCULAR MEDICINE (2022)

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Editorial Material Cardiac & Cardiovascular Systems

Bicuspid Aortopathy Toward Individualized Risk Assessment With Imaging Biomarkers COMMENT

Hector I. Michelena

JACC-CARDIOVASCULAR IMAGING (2022)

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Article Cardiac & Cardiovascular Systems

Wall Shear Stress Predicts Aortic Dilation in Patients With Bicuspid Aortic Valve

Andrea Guala et al.

Summary: This study assessed the predictive value of wall shear stress (WSS) for the growth rate (GR) of the ascending aorta (AAo) in patients with bicuspid aortic valve (BAV). The results showed a positive correlation between WSS and its circumferential component with AAo GR. Therefore, the evaluation of WSS may be helpful in the follow-up of these patients.

JACC-CARDIOVASCULAR IMAGING (2022)

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Article Cardiac & Cardiovascular Systems

Variation in management of paediatric isolated bicuspid aortic valve: current practice survey

Jesse M. Boyett Anderson et al.

Summary: This study describes the current follow-up, treatment, and intervention strategies for isolated bicuspid aortic valve in children with varying degrees of stenosis, regurgitation, and dilation, and compares them with the newly released appropriate use criteria. The results show deviations between current strategies and appropriate use criteria, highlighting the need for better delineation of the disease course, clarification of care recommendations, and wider adoption of guidelines.

CARDIOLOGY IN THE YOUNG (2022)

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Review Biochemistry & Molecular Biology

The Predictive Role of Plasma Biomarkers in the Evolution of Aortopathies Associated with Congenital Heart Malformations

Amalia Fagarasan et al.

Summary: Dilatation of the aorta is a condition with potential life-threatening events, and research is focusing on finding quantifiable biomarkers, such as MMPs and other candidate molecules, and their roles in diagnosis and prognosis. Many studies have found that certain biomarkers in serum are associated with different aortopathies, but research on pediatric data is still limited.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2022)

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Article Cardiac & Cardiovascular Systems

Natural history of bicuspid aortic valves and ascending aortic aneurysms: Aortic center experience

Christopher P. Bellaire et al.

Summary: The study found that for patients with ascending aortic aneurysms under 5.5 cm, regardless of valve type, conservative management with routine surveillance can achieve similar outcomes and a low rate of adverse events.

JOURNAL OF CARDIAC SURGERY (2022)

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Article Cardiac & Cardiovascular Systems

Cardiovascular Outcomes in Aortopathy GenTAC Registry of Genetically Triggered Aortic Aneurysms and Related Conditions

Kathryn W. Holmes et al.

Summary: The study compared the age distribution and probability of elective surgery and cardiovascular mortality among patients with different etiologies of aortopathy. The results showed differences in age and risk of surgery among patients with different etiologies.

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY (2022)

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Article Cardiac & Cardiovascular Systems

New England Congenital Cardiology Association Bicuspid Aortopathy Registry (NECCA BAR): A regional preventive cardiovascular care collaboration

Jonathan N. Flyer et al.

Summary: This study aims to improve regional collaboration for pediatric bicuspid aortopathy and investigate cardiovascular practice variation and short-term outcomes through the development of standardized medical management guidelines and patient care registry. Recruitment and data analysis are currently in progress.

PROGRESS IN PEDIATRIC CARDIOLOGY (2022)

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Article Cardiac & Cardiovascular Systems

Can 10 000 Healthy Steps a Day Slow Aortic Root Dilation in Pediatric Patients With Marfan Syndrome?

Elif Seda Selamet Tierney et al.

Summary: In this clinical intervention study on pediatric patients with Marfan syndrome, we found that a simple physical activity intervention can decrease the rate of aortic root dilation.

JOURNAL OF THE AMERICAN HEART ASSOCIATION (2022)

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Review Cardiac & Cardiovascular Systems

Bicuspid Aortic Valves: an Up-to-Date Review on Genetics, Natural History, and Management

Eman R. Rashed et al.

Summary: Bicuspid aortic valve (BAV) is a complex clinical entity with a wide spectrum of manifestations and unique hemodynamic interactions, requiring multidisciplinary management strategies. Further research is needed to fill knowledge gaps in both basic and clinical aspects of BAV.

CURRENT CARDIOLOGY REPORTS (2022)

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Review Cardiac & Cardiovascular Systems

Clinical implications of the biomechanics of bicuspid aortic valve and bicuspid aortopathy

Ali Fatehi Hassanabad et al.

Summary: This article presents the latest research on the biomechanics of bicuspid aortic valve (BAV), including definitions and classifications, mechanisms driving valve disease, and the impact of abnormal flow patterns on aortic dilatation. The role of cardiac magnetic resonance imaging in assessing and managing patients with BAV and bicuspid aortopathy is also discussed.

FRONTIERS IN CARDIOVASCULAR MEDICINE (2022)

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Article Cardiac & Cardiovascular Systems

2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines

Eric M. Isselbacher et al.

Summary: The 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease provides recommendations for clinicians on the diagnosis, genetic evaluation, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with various clinical presentations of aortic disease. The guideline emphasizes the importance of shared decision making and institutional interventional volume and multidisciplinary expertise in the care of patients with aortic disease.

CIRCULATION (2022)

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Article Medicine, General & Internal

Angiotensin receptor blockers and β blockers in Marfan syndrome: an individual patient data meta-analysis of randomised trials

Alex Pitcher et al.

Summary: In patients with Marfan syndrome, ARBs reduce the rate of increase in the aortic root Z score, including among those taking beta blockers. The effects of beta blockers are similar to those of ARBs. Combination therapy with ARBs and beta blockers provides a more significant reduction in the rate of aortic enlargement than either treatment alone.

LANCET (2022)

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Review Medicine, General & Internal

Patients with Bicuspid Aortopathy and Aortic Dilatation

Francesco Nappi et al.

Summary: Different imaging approaches play a crucial role in the diagnosis of BAV, with ultrasound scan being the primary step. Computed tomography and magnetic resonance help determine aortic vessel dimensions and hemodynamic alterations, leading to new classifications and surgical indications.

JOURNAL OF CLINICAL MEDICINE (2022)

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Review Cardiac & Cardiovascular Systems

Risk Stratification in Bicuspid Aortic Valve Aortopathy: Emerging Evidence and Future Perspectives

Alessandro Della Corte et al.

Summary: The current management of aortic dilatation associated with congenital bicuspid aortic valve is moving beyond dimensional parameters to investigate phenotypic criteria, flow-related metrics, and circulating biomarkers for risk stratification. Research is focusing on identifying severe root phenotypes and exploring the potential use of four-dimensional-flow magnetic resonance imaging and circulating molecules as predictors for aortopathy. This review highlights the latest progress in understanding the risk stratification of bicuspid aortic valve aortopathy.

CURRENT PROBLEMS IN CARDIOLOGY (2021)

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Article Cardiac & Cardiovascular Systems

Cost-effectiveness analysis of screening for first-degree relatives of patients with bicuspid aortic valve

Idit Tessler et al.

Summary: The study found that echocardiographic screening for first-degree relatives of BAV index cases is not only clinically important but also cost-effective and cost-saving. Sensitivity analysis supported the model's robustness, suggesting its generalization.

EUROPEAN HEART JOURNAL-QUALITY OF CARE AND CLINICAL OUTCOMES (2021)

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Article Cardiac & Cardiovascular Systems

Bicuspid aortic valve and aortopathy: novel prognostic predictors for the identification of high-risk patients

Luca Longobardo et al.

Summary: The study found that impairment of elastic properties of the ascending aorta in BAV patients, as assessed by 2D longitudinal strain, is an effective predictor of aortic complications.

EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING (2021)

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Article Cardiac & Cardiovascular Systems

Effect of Losartan or Atenolol on Children and Young Adults With Bicuspid Aortic Valve and Dilated Aorta

Jonathan N. Flyer et al.

Summary: This study evaluated the aortic growth rates in young patients with bicuspid aortopathy, and found that medical prophylaxis can reduce aortic dilation and absolute growth rates.

AMERICAN JOURNAL OF CARDIOLOGY (2021)

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Editorial Material Cardiac & Cardiovascular Systems

Bicuspid Aortopathy and Sports Clearance

Camden Hebson et al.

AMERICAN JOURNAL OF CARDIOLOGY (2021)

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Article Medicine, General & Internal

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

Anne-Sophie Sillesen et al.

Summary: The prevalence of bicuspid aortic valve among newborns in Copenhagen was 0.77%, with aortopathy present in 33% of cases. This suggests that BAV is likely a fetal malformation as indicated by the high rate of aortic disease among affected newborns.

JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION (2021)

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Article Physiology

Plasma Exosomal Mir-423-5p Is Involved in the Occurrence and Development of Bicuspid Aortopathy via TGF-β/SMAD2 Pathway

Hongqiang Zhang et al.

Summary: This study identified that plasma exosomal miR-423-5p regulates TGF-beta signaling by targeting SMAD2, thus exerting functions in the occurrence and development of BAV disease and its complication bicuspid aortopathy. These miRNAs may be involved in the pathogenesis of BAV disease.

FRONTIERS IN PHYSIOLOGY (2021)

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Article Cardiac & Cardiovascular Systems

Impact of Regular Physical Activity on Aortic Diameter Progression in Paediatric Patients with Bicuspid Aortic Valve

Emanuele Monda et al.

Summary: In this study of paediatric BAV patients, regular physical activity did not significantly affect aortic dilation progression over a 2-year period, indicating that the impact of exercise on aortic diameters in BAV patients remains unclear.

PEDIATRIC CARDIOLOGY (2021)

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Article Cardiac & Cardiovascular Systems

Multimodal Assessment of Vascular and Ventricular Function in Children and Adults With Bicuspid Aortic Valve Disease

Constance G. Weismann et al.

Summary: Patients with Bicuspid Aortic Valve (BAV) demonstrate increased proximal arterial stiffness and wave reflection, but no significant changes in Pulse Wave Velocity (PWV), common carotid intima media thickness (cIMT), or endothelial function when compared to controls. Diastolic function is mildly reduced in BAV patients, with moderate-strong correlations between arterial stiffness parameters, diastolic dysfunction, and age. Ascending aortic distensibility shows the strongest correlation with diastolic dysfunction in the BAV group, suggesting a different mechanism of arterial and cardiac stiffening compared to acquired heart diseases.

FRONTIERS IN CARDIOVASCULAR MEDICINE (2021)

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Review Biochemistry & Molecular Biology

Update in Biomolecular and Genetic Bases of Bicuspid Aortopathy

Alejandro Junco-Vicente et al.

Summary: Bicuspid aortic valve (BAV) associated with aortopathy is a common congenital heart disease that is complex and heterogeneous in nature. While recent studies have identified genetic mutations and explored molecular alterations involved, the pathophysiology of the disease is still not fully understood. Further research is needed to find therapeutic targets for prevention and treatment.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

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Article Pediatrics

Uptake of Screening and Recurrence of Bicuspid Aortic Valve and Thoracic Aortic Aneurysm Among At-Risk Siblings of Pediatric Probands

Daniel Miller et al.

Summary: This study reviewed the uptake of cardiac screening and recurrence of BAV and TAA in at-risk siblings, finding low screening uptake and high recurrence rates of BAV and TAA among siblings. This highlights the importance of increasing screening and early detection in at-risk siblings to optimize clinical management and improve health outcomes.

JOURNAL OF PEDIATRICS (2021)

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Article Cardiac & Cardiovascular Systems

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes

Hector Michelena et al.

Summary: This classification system recognizes three types of congenital bicuspid aortic valve condition and describes three types of aortopathy associated with it.

JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY (2021)

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Article Oncology

Aorta-specific DNA methylation patterns in cell-free DNA from patients with bicuspid aortic valve-associated aortopathy

Ashna Maredia et al.

Summary: The study demonstrated that elevated wall shear stress caused by abnormal flow hemodynamics in patients with bicuspid aortic valve-associated aortopathy undergoing surgery was associated with increased aortic wall cell death, supporting the use of aorta-specific cell-free DNA as a potential tool to identify aortopathy and stratify patient risk.

CLINICAL EPIGENETICS (2021)

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Article Cardiac & Cardiovascular Systems

Rapid ascending aorta stiffening in bicuspid aortic valve on serial cardiovascular magnetic resonance evaluation: comparison with connective tissue disorders

Alejandro Perez-Casares et al.

Summary: Aortic stiffness in BAV patients declines at a significantly steeper rate compared to CTD patients, particularly in the ascending aorta. However, the distensibility of the descending aorta declines at a similar rate in both groups, providing new insights into the differing pathogenesis of aortopathy in BAV and CTD patients.

JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE (2021)

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Article Cardiac & Cardiovascular Systems