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Advances in Aortic Stenosis

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What Is Bicuspid Aortic Stenosis?

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Your aortic valve is located at the junction of the left ventricle of your heart and your aorta, the largest blood vessel in your body. When your left ventricle contracts, your aortic valve opens, and blood is forced from your heart into your aorta and out to the rest of your body. When your left ventricle relaxes, your aortic valve closes, preventing blood from flowing backwards into your heart.

Most people have a tricuspid aortic valve composed of three leaflets, also called cusps or flaps, which allow it to open and close. Yet about 2% of the population have a bicuspid aortic valve, made up of only two leaflets. A bicuspid aortic valve may have difficulties fully opening and closing. A condition known as aortic stenosis can develop if the valve opening becomes narrowed.

What are the symptoms of bicuspid aortic stenosis?

A bicuspid aortic valve may not cause any symptoms early on. Occasionally, hearing a murmur during a doctor’s exam or discovering an infant or child is having difficulties feeding or gaining weight may prompt an early diagnosis.

My Aortic Stenosis Confession: Susan

More frequently, symptoms tend to appear years down the line or even into adulthood. Calcium deposits can build up on the abnormal leaflets of your aortic valve over time, making them stiffer and harder to open. Your heart has to work harder to pump blood through the narrow opening to get out to the rest of your body, and symptoms of aortic stenosis may appear. These include:

  • Fatigue
  • Shortness of breath
  • Chest pain
  • Heart palpitations
  • Fainting

What causes bicuspid aortic stenosis?

A bicuspid aortic valve is a congenital heart condition, meaning it’s something you’re born with. Research suggests bicuspid aortic valve disease may be related to a connective tissue disorder that can cause other problems with the circulatory system.

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How is bicuspid aortic stenosis treated?

To prevent complications, all patients with bicuspid aortic valves should be regularly monitored. Mild symptoms of aortic stenosis may be treated with lifestyle modifications and medications. If aortic stenosis becomes severe, your doctor may recommend surgery to treat the diseased valve and avoid further strain on your heart. These procedures include:

  • Balloon valvuloplasty: Generally reserved for infants and children or certain adults who aren’t candidates for surgery, this treatment requires a doctor to insert a long, flexible tube called a catheter into your arm or leg. The catheter has a collapsed balloon on its end and using medical imaging technology, it’s advanced to your aortic valve. The balloon is inflated to widen your aortic valve opening and then carefully deflated and removed.
  • Aortic valve repair: If the leaflets of your aortic valve are in good condition, they may be separated or reshaped to allow them to better open and close. This procedure is infrequently used, however.
  • Aortic valve replacement: Often the best option is to replace your non-functioning aortic valve with a replacement aortic valve. Tissue valves made from human or animal donors, or mechanical valves made of long-lasting materials, are both options for replacement aortic valves. Your own pulmonary valve (located between your right ventricle and pulmonary artery) may also be used as a replacement in certain cases. Your doctor will discuss which type of valve is best for you. You’ll also talk about what valve replacement procedure is most appropriate. Surgical aortic valve replacement (SAVR), which requires open-heart surgery, may be recommended for some, while transcatheter aortic valve replacement (TAVR) is emerging as a minimally-invasive option for many patients with an easier recovery than SAVR.

What are the potential complications of bicuspid aortic stenosis?

If your bicuspid aortic valve is unable to tightly close, blood can leak back into the left ventricle. This is called aortic valve regurgitation and can put further strain on your heart. Having a bicuspid aortic valve is also associated with having an enlarged aorta or developing an aortic aneurysm. If an aneurysm tears or bursts, it can be life-threatening.

The bottom line? Bicuspid aortic stenosis is a serious condition, but it can be managed. Stay on top of your health by working closely with your doctor to monitor for changes along the way. It’s a life-long commitment, but it’s worth it.

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2021 Mar 16
View All Advances in Aortic Stenosis Articles
THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
  1. Aortic Valve Stenosis. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/aortic-stenosis/symptoms-causes/syc-20353139
  2. Bicuspid Aortic Valve. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/bicuspid-aortic-valve/cdc-20385577
  3. Bicuspid Aortic Valve. MedlinePlus. https://medlineplus.gov/ency/article/007325.htm
  4. Evolut Low Risk Bicuspid Study: Favorable Safety With TAVR in Patients With Bicuspid Valve. American College of Cardiology. https://www.acc.org/latest-in-cardiology/articles/2020/03/24/16/41/sun-8am-tavr-patients-severe-bicuspid-aortic-valve-stenosis-acc-2020
  5. Aortic Valve Disease. University of Wisconsin Madison School of Medicine and Public Health. https://www.uwhealth.org/heart-cardiovascular/aortic-valve-stenosis/11089