What is aortic valve stenosis?

Aortic valve stenosis occurs when the aortic valve is blocked or narrow and does not work correctly. This one-way valve controls blood flow from the left ventricle inside the heart to the aorta, the main blood vessel that brings blood to the body. When the aortic valve does not work properly, the heart must work harder to pump blood out to the body. Over time, this can weaken the heart.

Aortic valve stenosis can range from mild to severe. As the child gets older, the condition can get worse as the heart has to work harder to pump blood.

  • Subvalvular – The narrowing is below the aortic valve
  • Valvular – The narrowing is at the aortic valve with fusion of valve leaflets
  • Supravalvular – The narrowing is above the aortic valve

Aortic stenosis results in a muscle enlargement, or compensatory ventricular hypertrophy, of the left ventricle. The degree of hypertrophy is generally related to the severity of the stenosis.

What are the symptoms of aortic valve stenosis?

Many children with mild to moderate aortic valve stenosis don’t have any symptoms at first. The only symptom they may have is a heart murmur. This is an extra heart sound your child’s doctor can hear using a stethoscope.

Over time, the aortic valve stenosis may progress and cause symptoms including:

  • Shortness of breath
  • Chest pain
  • Feeling tired
  • Fainting 

What causes aortic valve stenosis?

In most cases, aortic valve stenosis is a condition a baby is born with. It occurs when there is a problem with the way the valve is formed. These problems may include:
  • An aortic valve that is too narrow
  • An aortic valve that has only two parts (leaflets) instead of three
  • Leaflets that are stuck together or thicker than normal and do not open all the way

Aortic valve stenosis can also be caused by rheumatic fever. This can happen if scar tissue forms on the aortic valve. 

How is aortic valve stenosis treated?

Your child’s treatment will depend on their age and how severe their condition is. Some children with mild aortic valve stenosis may not need any treatment. Instead, your child’s doctor may track their condition over time. Your care team at Arkansas Children’s is experienced in treating aortic valve stenosis and will work with you to come up with the best treatment option for your child. Treatments may include:

  • Balloon valvuloplasty is the most common treatment for aortic valve stenosis. It is a minimally invasive procedure using a narrow, flexible tube called a catheter. During the procedure, the doctor inserts the catheter into a blood vessel in the groin. Then, using tiny cameras and tools, guides the catheter into the heart. Once it reaches the aortic valve, a deflated balloon on the end the catheter is inflated to stretch open the aortic valve. Then the balloon is deflated and removed.
  • Your child may need a valve repair or replacement if their condition is more severe. It may also be a next step if your child has had a balloon valvuloplasty and the valve has become narrow again. Repairing the valve to improve its function is the first choice, if at all possible. If repair is not an option, your child’s doctor may discuss replacing the valve with an artificial or donor valve.
  • While the surgical techniques for each of these valvular narrowings are different, the objective is the same: to relieve the obstruction. Surgical treatment for aortic stenosis involves making an incision through the breastbone (sternotomy). A machine that allows the heart and lungs to rest (cardiopulmonary bypass) is used to allow the surgery team to repair the area of concern.
    • Subvalvular Stenosis (below the valve) – The aorta is opened just above the valve, and the obstruction is removed from below the valve (subvalvular ring of tissue).
    • Valvular Stenosis (at the valve) – The aorta is opened in a similar fashion, however the valve itself is addressed. Excision of the fused leaflet(s) is performed with or without commissuroplasty.
    • Supravalvular Stenosis (above the valve) – An incision is made over the affected area and a patch is sewn in place to enlarge the narrowing (aortoplasty).

After an aortic stenosis repair, monitoring in the postoperative period will include invasive lines, such as an arterial line and central venous line, to monitor blood pressure and deliver medications. Medications may be needed to control hemodynamics (nitroprusside or nicardipine), provide sedation and maintain hydration during recovery. Perfusion is monitored by pulse oximetry and NIRS (near-infrared spectroscopy) probes. Depending on various factors specific to each individual, the breathing tube (endotracheal tube) may or may not still be present after surgery. Pacing wires, which can assist with any rhythm issues that may arise in the postoperative period, will be placed in the operating room. Pacing wires are typically removed 1-2 days after surgery if not needed. Chest tubes will be present to remove air, blood or fluid from around the heart or lungs. These tubes will be removed in the ICU as soon as possible, typically the next day.

Length of stay can vary but typically ranges from 1-3 weeks, depending on the degree of stenosis and associated surgical repair required. 

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