Unicuspid Aortic Valve as a Variant of Bicuspid Aortic Valve

Definition and Classification

• Abnormal valve development with reduced number of cusps compared to the normal tricuspid aortic valve is a characteristic of both BAV and UAV, affecting 1-2% of the population for BAV, while UAV is much rarer 1, 2

Evidence Supporting UAV as a BAV Variant

• The European Society of Cardiology recommends considering UAV as an extreme variant of BAV, with similar demographics and clinical findings as BAV patients 2

Clinical Implications

• Both BAV and UAV are associated with aortic valve dysfunction, thoracic aortic dilatation and aneurysm formation, and risk of aortic dissection, according to the American Heart Association 3, 4

Management Considerations

• The American College of Cardiology recommends that all patients with abnormal aortic valve morphology, including BAV or UAV, should have both the aortic root and ascending thoracic aorta evaluated for evidence of aortic dilatation 3
• First-degree relatives of patients with BAV or UAV should be evaluated for the presence of abnormal valve morphology and asymptomatic thoracic aortic disease, as suggested by the American Heart Association 3