Overview
Bicuspid aortic valve (BAV) is a congenital heart defect occurring in 1-2% of the population, characterized by the presence of two valve leaflets instead of three. It predisposes individuals to valvular complications such as aortic stenosis and regurgitation, as well as aortopathy including aortic root dilation and dissection 14.Diagnosis
Echocardiography: Essential for initial diagnosis, assessing valve function, and aortic root dimensions 12.
Z-score Calculation: Used to evaluate aortic root dilation in pediatric and adult patients 2.
Genetic Screening: Recommended for first-degree relatives due to suspected heritability 2.
Imaging Modalities: CT or MRI for detailed aortic anatomy in complex cases 1.Management
Surgical Intervention: Indicated for severe aortic stenosis, significant aortic regurgitation, or aortic dilation ≥45 mm 14.
Aortic Root/Ascending Aorta Replacement: Recommended based on specific diameter thresholds and clinical presentation 1.
Repair Techniques: Novel repair methods using autologous pericardium for calcified BAV show promising early results 3.
Multidisciplinary Approach: Emphasizes involvement of experienced surgeons and multidisciplinary aortic teams (MATs) 1.Special Populations
Pediatrics: Regular echocardiographic screening of first-degree relatives is feasible and effective 2.
Pregnancy: Specific management guidelines are not detailed in provided abstracts; individualized risk assessment is crucial 4.
Elderly: Considerations for surgical risk and valve durability are paramount in elderly patients with BAV 1.Key Recommendations
Screen First-Degree Relatives: Perform echocardiographic assessment for first-degree relatives of BAV patients 2 (Evidence: Moderate).
Aortic Root/Ascending Aorta Replacement: Consider surgical replacement when aortic dimensions meet specific thresholds (e.g., ≥45 mm) 1 (Evidence: Strong).
Multidisciplinary Team Involvement: Engage experienced surgeons and multidisciplinary aortic teams for optimal management 1 (Evidence: Expert opinion).References
1 Verma R, Cohen G, Colbert J, Fedak PWM. Bicuspid aortic valve associated aortopathy: 2022 guideline update. Current opinion in cardiology 2023. link
2 Massardier C, Desroches F, Singbo N, Côté JM, Drolet C, Houde C et al.. First-Degree Relatives Screening of Patients with Bicuspid Aortic Valve: Effectiveness and Feasibility in Pediatric Cardiology Daily Practice. Pediatric cardiology 2020. link
3 Urbanski PP. Repair of the calcified bicuspid aortic valve. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2011. link
4 Friedman T, Mani A, Elefteriades JA. Bicuspid aortic valve: clinical approach and scientific review of a common clinical entity. Expert review of cardiovascular therapy 2008. link