Causes and Diagnoses

Causes and Diagnoses of Bicuspid Aortic Valve

While it is the most common congenital heart disorder, the cause of bicuspid aortic valve (BAV) is not completely clear.

  • BAV develops early in fetal development and is present when a baby is born
  • Many patients will live their whole lives unaware they have BAV
  • BAV symptoms can start at any time

Diagnosing BAV

One of the earliest indicators of BAV may be a heart murmur. A heart murmur is an abnormal sound caused by the turbulent flow of blood over a diseased heart valve.

Hearing a heart murmur may trigger a cardiologist to order an echocardiogram (ECHO) to determine if you have BAV. An ECHO is a painless test that uses sound waves to create a moving picture of the heart. It is the most accurate way of diagnosing BAV.

Follow-up testing and long-term care

If you are diagnosed with BAV, you should receive ongoing care from a cardiologist with experience in heart valve disease. A cardiologist will carefully monitor any changes in your heart function, valves and aorta over time.

Cardiologists with expertise in heart valve disease will use advanced imaging tests to assess your heart’s condition over time. Because the time between diagnosis of BAV and the need for potential surgery is different for everyone, it is important to be seen by a cardiologist for follow-up.

Follow-up care allows your cardiologist to monitor your BAV, aorta and overall heart function by using a variety of advanced imaging tests including ECHO, CT scan and MRI.

Even after surgical replacement of the BAV, you may be at risk for future problems with your aorta. Because of this, it is important to continue follow-up care with your cardiologist, as they will create a plan of care to ensure that you are monitored appropriately.

Family Screening

If a family member has been diagnosed with BAV there is a nine percent chance of additional family members having BAV as well. If a family member has been diagnosed with BAV, The Martha and Richard Melman Family Bicuspid Aortic Valve Program at Northwestern Medicine as well as the American College of Cardiology/American Heart Association recommends that family members be screened for BAV.

Current recommendations are for all first-degree relatives (parents, siblings, children) of someone diagnosed with BAV to be screened. Early diagnosis of BAV allows the patient to be followed prior to the disease causing symptoms and complications.

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