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 with Hypoplasia

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