• Most common congenital heart disease abnormality involving 1-2% of the population.
• Strong risk factor for developing early aortic stenosis, aortic regurgitation and is associated with aortopathy such as aortic dissection and aneurysm.
• Should screen all first degree relatives.

Types

• Fusion of right and left leaflet most common usually accounts for 80% of cases.
• Right and non-coronary leaflet the next most common usually 20% of cases.
• Fusion of Left with non-coronary is very rare.
• Unicuspid valve is also very rare.

Role of Echo

• Crucial for diagnosis and characterizing the leaflets involves.
• Important for identifying associated diseases such as aortic aneurysms, associated aortic stenosis or regurgitation.
• Bicuspid valves are best visualized in the short axis view as this allows for the best characterization of the involved leaflets. Long axis views may give clues to the presence of a bicuspid valve as the valve closure line may be eccentric.

Example 1 (Right and Left Leaftlet Fusion)

• This Long axis view focused on the ascending aorta, though subtle there is certainly a question of the valve closure line is eccentric. This is often the first clue there may be a bicuspid valve, often confirmed in short axis.

• This is a short axis view at the level of the aortic valve and an example of the most common bicuspid valve with fusion of right and left leaflets.