Bicuspid Aortic Valve

Bicuspid aortic valve is one of the most common congenital lesions of the cardiovascular system.  In this entity only two aortic valve cusps are developed. Patients with BAV commonly have aortic narrowing and regurgitation of variable severity.

The cause is congenital.  Most are usually isolated mild lesions but are prone to progressive calcification, and advancing aortic stenosis later in life. Sometimes it is associated with other left sided lesions including coarctation of the aorta, and congenital mitral stenosis.  In the most severe forms severe stenosis and atresia are present.  The full development of all the structures in the heart depends on adequate flow through them.  Thus if there is limitation of flow at one level there are often maldevelopments down stream.

With time bicuspid aortic valve becomes more fibrotic and  the gradient increases and the left ventricle hypertrophies to accommodate the increased pressure and work load.  The structural distortion of the valve predisposes it to infection and BAV therefore can be complicated by bacterial endocarditis.

Aneurysmal disease of the ascending aorta is thought to relate to an  aortopathy

Other syndromes associated with bicuspid aortic valve and ascending aortic aneurysm include Turner syndrome (bicuspid valve present in approximately 30 percent), Loeys-Dietz syndrome

Other lesions coarctation of the aorta, supravalvular aortic stenosis, subvalvular aortic stenosis, ventricular septal defect  patent ductus arteriosus, and sinus of Valsalva aneurysm

A bicuspid aortic valve is present in approximately 15 to 20 percent of patients with sinus of Valsalva aneurysms.

Heart murmurs result from either regurgitation or stenosis and are best diagnosed by echocardiography

Surgery with replacement of the valve is indicated when the pressure gradient become high, usually with gradients that are greater than 50-60mmHg across the valve.

Bicuspid Aortic Valve
Fusion of the inter-coronary commissure
Fusion of the right and left coronary leafletas shown here is the most common (70 to 85 percent)
Ashley Davidoff

Bicuspid Aortic Valve with Hypoplasia

Hypoplastic Annulus, Bicuspid Aortic Valve and Malposition of Coronary Ostium
15049
Ashley Davidoff MD
Bicuspid Aortic Valve Caused by Fusion of Two Commissures Between the Right and Non Coronary Cusp
07951b
Ashley Davidoff MD
This gray scale echo of the heart showing a short-axis aorta left atrial view, and demonstrating the aortic valve with two cusps. The patient has a diagnosis of bicuspid aortis valve
33169b Ashley Davidoff
Keywords:
cardiac heart echo aorta bicuspid aortic valve congenital imaging cardiac echo
CT of the aortic valve in sagittal plane
demonstrating the aortic valve with two cusps. The patient has a diagnosis of bicuspid aortis valve
Ashley Davidoff
Bicuspid Aortic Valve

The Common Vein Copyright 2007

Ashley Davidoff MD

 

Bicuspid Aortic Valve (BAV)

Bicuspid aortic valve is one of the most common congenital lesions of the cardiovascular system.  In this entity only two aortic valve cusps are developed. Patients with BAV commonly have aortic narrowing and regurgitation of variable severity.

The cause is congenital.  Most are usually isolated mild lesions but are prone to progressive calcification, and advancing aortic stenosis later in life. Sometimes it is associated with other left sided lesions including coarctation of the aorta, and congenital mitral stenosis.  In the most severe forms severe stenosis and atresia are present.  The full development of all the structures in the heart depends on adequate flow through them.  Thus if there is limitation of flow at one level there are often maldevelopments down stream.

With time bicuspid aortic valve becomes more fibrotic and  the gradient increases and the left ventricle hypertrophies to accommodate the increased pressure and work load.  The structural distortion of the valve predisposes it to infection and BAV therefore can be complicated by bacterial endocarditis.

Heart murmurs result from either regurgitation or stenosis and are best diagnosed by echocardiography

Surgery with replacement of the valve is indicated when the pressure gradient become high, usually with gradients that are greater than 50-60mmHg across the valve.

Bicuspid Aortic Valve with Hypoplastia

 

Hypolastic Annulus, Bicuspid Aortic Valve and Malposition of Coronary Ostium
15049 Davidoff MD

Isolated Congenital BAV

Bicuspid Aortic Valve Caused by Fusion of Two Commissures Between the Right and Non Coronary Cusp
07951b Davidoff MD

 

Bicuspid Aortic Valve
This gray scale echo of the heart showing a short-axis aorta left atrial view, and demonstrating the aortic valve with two cusps. The patient has a diagnosis of bicuspid aortis valve which is a congenital condition. Courtesy Philips Medical Systems 33169 code cardiac heart echo aorta bicuspid aortic valve congenital imaging cardiac echo

 

Normal and Thickened Aortic Valve over Time
07953c02 heart cardiac aorta aortic valve fx normal fx thickened fx bicuspid aortic valve fx calcified fx calcification fusion of the intercoronary commisures grossanatomy grosspathology Davidoff MD b Courtesy Henri Cuenoid MD
Aortic Stenosis – Doming of the Valve
07969bW.802 heart cardiac aorta aortic valve fx thickening of the aortic valves LVH left ventricular hypertrophy post stenotic dilatation of the ascending aorta turbulence eccentric jet doming of the aortic valve AV AS aortic stenosis Davidoff art

 

Aortic Stenosis – Turbulence Shown by MRI
This series of coronal MRI images of the aortic valve (a-f) show phases from diastole (a) through systole (b,c,d,e) with a narrow (b,c) and then turbulent jet, (d,e) back to diastole (f) Image g shows a thickened valve, while the short axis of the LV (h) shows LV hypertrophy. The plain film of h and i highlight the calcific nature of the valve. The diagnosis is aortic valve stenosis. Courtesy Scott TSai MD  code cardiac heart aortic valve AS LVH calcification calcified imaging radiology MRI CXR plain film 38871c01