Aortic Stenosis

What is aortic stenosis?

Aortic stenosis is a narrowing at, below, or above the level of the aortic valve, which can lead to reduction in blood flow to the body.

Types of aortic stenosis

Valvular aortic stenosis

Valvular aortic stenosis is narrowing at the level of the valve and is most often caused by a bicuspid aortic valve (valve has two leaflets, rather than three). Most often, bicuspid aortic valves do not cause issues before adulthood.

Critical aortic stenosis

Critical aortic stenosis is a pinhole opening in the aortic valve found in newborns. It is usually associated with a small aorta (large blood vessel that comes from the left ventricle and supplies blood to the body). It can also be associated with small left ventricle, mitral valve, left ventricular outflow tract (the path out of left ventricle), or ventricular septal defect (hole between the bottom chambers of the heart).

Supravalvular aortic stenosis

Supravalvular aortic stenosis is a narrowing in the aorta just above the aortic valve. There can also be additional narrowing in the aorta. Supravalvular aortic stenosis is often associated with Williams Syndrome.

Subvalvular (subaortic) aortic stenosis

• • Discrete: a localized narrowing below the aortic valve (more common)
    • Two thirds of these children also commonly have ventricular septal defect (a hole between the bottom chambers of the heart) and coarctation of the aorta (narrowing in large artery that comes off heart and supplies blood to the body)
    • One third of the time, it occurs as an isolated defect, or familial (genetic condition)
    • Can also be associated with prior surgery such as ventricular septal defect closure or pulmonary artery banding
  • Tunnel-like narrowing: long narrowing below the level of the valve
    • Often associated with a small ascending aorta and the aortic valve annulus (ring around the aortic valve), also thickened aortic valve leaflets (flaps of tissue that help to close the valve).
    • Can also be associated with idiopathic hypertrophic subaortic stenosis (IHSS), which is a primary problem with the heart muscle.

Subaortic membrane

Subaortic membrane is a “film” that forms under the aortic valve and can make it difficult for blood to leave the heart and reach the body.

Diagnosing aortic stenosis

Aortic stenosis is diagnosed through echocardiography (ultrasound of the heart). Sometimes a murmur will be heard, prompting the testing. Early diagnosis and treatment is very important, especially in severe cases of aortic stenosis.

Signs and symptoms of aortic stenosis

• Severe to critical: Poor feeding, poor perfusion (blood flow) to the body, fast breathing, fluid buildup in the lungs, weak pulses, cool and pale skin.
• Older children with severe aortic stenosis may experience chest pain when they exercise, shortness of breath, or fainting.
• Less severe aortic stenosis can cause little to no symptoms in older children, though they may experience difficulty with exercise.
  
  

Testing for aortic stenosis

• Pulse oximetry: a way to monitor the oxygen content of the blood via a light probe placed on your baby’s hand or foot. This test is not painful.
• Electrocardiogram (ECG): a visual representation of the heart's electrical activity captured via monitors placed on the skin. This test is not painful.
• Echocardiogram (echo): an ultrasound of the heart that evaluates the structure and the function of the heart by using sound waves. Still and moving pictures of the heart structures, heart valves, and heart function are recorded for review by a cardiologist. This test is not painful.
• Chest X-ray: a test that uses a small amount of radiation to create an image (or picture) within the chest to include the heart, lungs, blood vessels and bones. This test is not painful.
  
  

Aortic stenosis treatment options

Medical management

• In infants with severe degree of aortic stenosis, a medication called prostaglandins will be used to keep the ductus arteriosus open, so that blood can reach the body.
• This medication is given through an IV as a continuous infusion.
• Children with less severe aortic stenosis can be managed on an outpatient basis by a cardiologist.

Interventional cardiology

• Cardiac catheterization: a procedure where a catheter (small tube) is inserted into your child’s heart through a large vein or artery in the leg to take pictures and pressure measurements. There may be some soreness at the insertion site following this procedure.
• The interventional cardiologist will use a balloon to relieve the aortic stenosis by opening the narrowed area during the cardiac catheterization procedure.

Surgical management

• Surgery may be necessary if the balloon procedure was unsuccessful or caused a significant amount of leakage of the valve.
• Surgery may also be necessary if the valve has become too narrow or leaky, or if the heart muscle shows changes. Surgery may include repair or replacement of the aortic valve, or fixing the narrowing that has occurred above or below the valve.
• See Children’s Mercy surgical outcomes.

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