Rapid Review·Cardiovascular
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Valvular Heart Disease
T1Must knowBicuspid Aortic Valve
Focus on
The most common congenital heart defect, found in 1–2% of people and in Turner syndrome: an ejection click and early calcific stenosis in the 40s–60s, sometimes regurgitation, and an aortopathy that dilates the ascending aorta. Echo the valve, image the aorta, screen the relatives.
Key takeaways
What it is
What this shows
Bicuspid aortic valve
two of the three aortic leaflets fuse in utero.Turner syndrome
strongly associated.Clinical features
Early aortic stenosis
the valve calcifies early, so aortic stenosis appears after age 40 (versus the 50s and 60s for wear-and-tear disease).Aortic regurgitation
the less common presentation.Ascending aortic aneurysm
a raised risk at a relatively young age.Diagnosis and management
Same as the single-valve lesions
as for Aortic Stenosis and Aortic Regurgitation, confirm with transthoracic echocardiography, and replace the valve by the same criteria.A 46-year-old man has exertional dyspnea, a harsh systolic ejection murmur radiating to the carotids and an early ejection click. What is the likely valve, which syndrome is it strongly associated with, and what else must be imaged?
A bicuspid aortic valve with early calcific aortic stenosis (stenosis after 40, not the 50s and 60s of wear-and-tear disease). It is strongly associated with Turner syndrome. Image the ascending aorta, because the aneurysm risk is raised.
How it's tested
A 48-year-old man with exertional dyspnea, a harsh systolic murmur radiating to the carotids and an ejection click: bicuspid aortic valve with calcific stenosis — echocardiogram; surgical AVR when severe, with a look at the ascending aorta.
A 16-year-old girl with short stature, webbed neck and a systolic ejection click: Turner syndrome — bicuspid valve, and check for coarctation.
Bicuspid valve with an ascending aorta of 5.2 cm and a father who died of dissection: elective ascending aortic replacement.
Should a patient with an uncomplicated bicuspid valve take antibiotics before dental work: no — prophylaxis is reserved for prosthetic valves, prior endocarditis and cyanotic congenital disease.
Go deeper
Guidelines: 2020 ACC/AHA Valvular Heart Disease Guideline · 2022 ACC/AHA Aortic Disease Guideline
Related Step 2 pages: Aortic Stenosis, Aortic Regurgitation, Thoracic Aortic Aneurysm, Coarctation of Aorta, Infective Endocarditis
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