Bicuspid aortic valve (BAV) is a form of congenital heart disease (the person is born with it). With BAV, the heart valves in the aorta (the blood vessel that takes blood away from the heart to the body) are not formed right. A person with BAV has only 2 leaflets instead of three and the valve leaflets are often thickened. This can result in the block of blood flow across the valve (aortic stenosis) and/or valve leakage (aortic valve regurgitation).

From our experience at least 1/3 of patients with BAV will eventually develop complications. Many patients with BAV do not develop significant problems until well into adulthood. The most common problem in BAV patients is aortic dilatation and/or dissection. At this point, we do not know on who or why aortic dilatation or dissection occurs.It is unclear whether the enlargement is because of abnormal blood flow patterns, as a result of the shape of the bicuspid valve, or whether it is because the way the aortic valve and/or vessel is formed. In other words, the abnormal shape of the aortic valve may cause blood to flow in a different way than it normally would, causing damage to the aorta as blood leaves the heart. There may be a problem with the way the aortic valve connects to the aorta, which causes the aorta to get larger or break down over time. It is also possible that the wall of the aorta in patients with BAV is weaker than it would be in patients without BAV. At this point, we do not know. It is believed by the investigators that if we can determine why the aorta gets larger or tears, we can minimize the effects or prevent them altogether.

This study will collect blood and cardiac MRI images from forty-five (45) patients at Children's Healthcare of Atlanta Egleston. There will be a study group (patients with BAV) and a control group of patients (patients scheduled for a cardiac MRI but without BAV).

All enrolled patients will have blood drawn by nursing staff from a peripheral vein and collected in tubes for testing the day of their MRI scan. This test is called a plasma matrix metalloproteinase level. It is believed that patients who have bicuspid aortic valves and dilated aortas have high plasma levels of this protein. This study will compare the MRI images and plasma matrix protein levels of all the patients participating in the study.