Bicuspid Aortic Valve Aortopathy

Summary
Bicuspid Aortic Valve (BAV) Aortopathy is a condition affecting 0.5-2% of the population, with higher prevalence among males. BAV can lead to various complications, including aneurysms and dissections, particularly influenced by factors such as valve morphology, wall shear stress, and genetic predisposition.

Key Information

• Epidemiology: BAV is observed in 0.5-2% of the population, with 6-10% of first-degree relatives showing related conditions, such as aortic or mitral valve disease.
• Risks: There’s a significant risk of aortic aneurysms (80-85%) and dissections (<5%), with aortic root dilation observed more commonly in males.
• Valve Morphology: BAV morphology impacts disease progression. Siever’s classification categorizes BAV into types based on leaflet orientation and cusp fusion.
• Genetic Predisposition: Several genetic disorders are linked to BAV, including Marfan syndrome, Turner syndrome, Loeys-Dietz syndrome, and others, which increase the risk of aortopathy.
• Management and Surveillance: Regular monitoring through imaging (CT/MR > TTE) is recommended for patients with aortic dilation. Surgical intervention is considered when aneurysms exceed specific size thresholds.

Created by: Dr. Najah Khan
Edited by: Dr. Evelyn Song

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