Learn About Bicuspid Aortic Valve

What is the definition of Bicuspid Aortic Valve?

A bicuspid aortic valve (BAV) is an aortic valve that only has two leaflets, instead of three.

The aortic valve regulates blood flow from the heart into the aorta. The aorta is the major blood vessel that brings oxygen-rich blood to the body.

Bicuspid aortic valve
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What are the alternative names for Bicuspid Aortic Valve?

Bicommissural aortic valve; Valvular disease - bicuspid aortic valve; BAV

What are the causes of Bicuspid Aortic Valve?

The aortic valve allows oxygen-rich blood to flow from the heart to the aorta. It prevents the blood from flowing back from the aorta into the heart when the pumping chamber relaxes.

BAV is present at birth (congenital). An abnormal aortic valve develops during the early weeks of pregnancy, when the baby's heart is developing. The cause of this problem is unclear, but it is the most common congenital heart defect. BAV often runs in families.

A BAV may not be completely effective at stopping blood from leaking back into the heart. This leakage is called aortic regurgitation. The aortic valve may also become stiff and not open up. This is called aortic stenosis, which causes the heart to pump harder than usual to get blood through the valve. The aorta may become enlarged with this condition.

BAV is more common among males than females.

A BAV often exists in babies with coarctation of the aorta (narrowing of the aorta). BAV is also seen in diseases in which there is a blockage to blood flow on the left side of the heart.

What are the symptoms of Bicuspid Aortic Valve?

Most of the time, BAV is not diagnosed in infants or children because it causes no symptoms. However, the abnormal valve can leak or become narrow over time.

Symptoms of such complications may include:

  • Baby or child tires easily
  • Chest pain
  • Difficulty breathing
  • Rapid and irregular heartbeat (palpitations)
  • Loss of consciousness (fainting)
  • Pale skin

If a baby has other congenital heart problems, they may cause symptoms that will lead to the discovery of a BAV.

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What are the current treatments for Bicuspid Aortic Valve?

The infant or child may need surgery to repair or replace a leaky or narrowed valve, if complications are severe.

A narrowed valve can also be opened through cardiac catheterization. A fine tube (catheter) is directed to the heart and into the narrow opening of the aortic valve. A balloon attached to the end of the tube is inflated to make the opening of the valve larger.

In adults, when a bicuspid valve becomes very leaky or very narrowed, it may need to be replaced. This used to only be able to be done using open heart surgery, but can now sometimes be done via catheters.

Sometimes the aorta may also need to be repaired if it has become too wide or is too narrow.

Medicine may be needed to relieve symptoms or prevent complications. Medicines may include:

  • Drugs that lower the workload on the heart (beta-blockers, ACE inhibitors)
  • Drugs that make the heart muscle pump harder (inotropic agents)
  • Water pills (diuretics)
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What is the outlook (prognosis) for Bicuspid Aortic Valve?

How well the person does depends on the presence and severity of complications of BAV.

The presence of other physical problems at birth also can affect how well a person does.

Most babies with this condition have no symptoms, and the problem is not diagnosed until they are adults. Some people never find out that they have this problem.

What are the possible complications of Bicuspid Aortic Valve?

Complications of BAV include:

  • Heart failure
  • Leakage of blood through the valve back into the heart
  • Narrowing of the valve's opening
  • Infection of the heart muscle or aortic valve
When should I contact a medical professional for Bicuspid Aortic Valve?

Contact your child's provider if your baby:

  • Has no appetite
  • Has unusually pale or bluish skin
  • Seems to tire easily
How do I prevent Bicuspid Aortic Valve?

BAV runs in families. If you know of this condition in your family, speak to your provider before becoming pregnant. There is no known way to prevent the condition.

What are the latest Bicuspid Aortic Valve Clinical Trials?
Transcatheter Aortic Valve Replacement (TAVR) With Medtronic TAVR System in Patients With Severe Bicuspid Aortic Valve Stenosis and at Low Predicted Risk of Mortality With Surgical Aortic Valve Replacement (SAVR)

Summary: The objective of the trial is to evaluate the procedural safety and efficacy of the Medtronic TAVR system in patients with bicuspid aortic anatomy and severe aortic stenosis at low risk for SAVR

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Transcatheter Aortic Valve Replacement For Patients With Bicuspid Aortic Stenosis (Type 0) Using Down Sizing Strategy Compared With Standard Sizing Strategy (HANGZHOU Solution): A Prospective, Multicenter, Randomized Controlled Trial

Summary: To compare down sizing strategy versus annular sizing strategy technique (control group) in Type 0 bicuspid aortic stenosis (AS) patients undergoing transcatheter aortic valve replacement (TAVR) with self-expanding valves (SEVs): a randomized superiority trial

What are the Latest Advances for Bicuspid Aortic Valve?
Repair of a stenotic bicuspid aortic valve by extensive leaflet shaving and commissuroplasty.
Current controversies in aortic valve-preserving surgery.
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Single-Center Experience With Aortic Coarctation Stenting in Adult Patients.
Who are the sources who wrote this article ?

Published Date: May 08, 2022
Published By: Michael A. Chen, MD, PhD, Associate Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, WA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

Borger MA, Fedak PWM, Stephens EH, et al. AATS consensus guidelines on bicuspid aortic valve-related aortopathy: full online only version. J Thorac Cardiovasc Surg. 2018;156(2):e41-74. PMID: 30011777 pubmed.ncbi.nlm.nih.gov/30011777/.

Braverman AC, Cheng A. The bicuspid aortic valve and associated aortic disease. In: Otto CM, Bonow RO, eds. Valvular Heart Disease: A Companion to Braunwald's Heart Disease. 5th ed. Philadelphia, PA: Elsevier; 2021:chap 11.

Fraser CD, Cameron DE, McMillan KN, Vricella LA. Heart disease and connective tissue disorders. In: Ungerleider RM, Meliones JN, McMillian KN, Cooper DS, Jacobs JP, eds. Critical Heart Disease in Infants and Children. 3rd ed. Philadelphia, PA: Elsevier; 2019:chap 53.

Gentile F, Castiglione V, De Caterina R. Coronary Artery Anomalies. Circulation. 2021;144(12):983-996. Epub 2021 Sep 20. PMID: 34543069 pubmed.ncbi.nlm.nih.gov/34543069/.

Leon MB, Mack MJ. Transcatheter Aortic Valve Replacement. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022: chap 74.