Bicuspid Aortic Valve

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Presentation :

Most common congenital heart abnormality
Can cause both aortic valve stenosis (up to 75%) and regurgitation (2-10%)
May co-occur with other cardiovascular structural abnormalities, including: aortic coarctation, aneurysm of the sinuses of Valsalva, PDA, and aortic aneurysm and dissection

The ascending aorta and aortic arch should be examined for aortopathy with TTE
- First-degree relatives of patients with both bicuspid aortic valve AND aortopathy should be screened with echocardiography.
- The ascending aortic diameter should be assessed at least annually by echocardiography if the aortic root or ascending aorta dimension is >4.5 cm.

Indications for stenotic valve replacement are the same as for tricuspid valve Aortic Stenosis
Valve replacement is also indicated when Aortic Regurgitation is clinically significant: symptomatic HF or asymptomatic LVEF <50%.
Aortic root repair is indicated if:
- severe AS or regurgitation requiring replacement and aortic diameter >4.5 cm
- aortic root diameter >5 cm with an additional risk factor for dissection
- aortic root diameter >5.5 cm without risk factors