Aortic Stenosis

What is Aortic Stenosis?

The aortic valve is one of four valves in the heart. Each valve is responsible for keeping the blood flowing in one direction; the aortic valve keeps blood flowing from the left ventricle to the aorta. In a normal heart, the aortic valve has three leaflets, or flaps, that keep the blood flowing one-way. In a child with aortic stenosis, the valve is too narrow or is blocked, making it more difficult for the leaflets to allow blood flow to the aorta.

Aortic stenosis can be congenital, meaning the valve didn’t develop properly before the child was born. In most cases, there is no known cause, though it can sometimes be due to a genetic problem. Congenital aortic stenosis also occurs more often in boys. Aortic stenosis can also be acquired later in childhood, usually due to an untreated strep infection.

Symptoms of Aortic Stenosis

Aortic stenosis can range from mild to severe, depending on the level of blood flow obstruction. The symptoms of aortic stenosis will vary depending on the severity of the condition and your child’s age. A child with mild aortic stenosis may have few, if any symptoms. In more severe cases of aortic stenosis, symptoms may include:

• Cyanosis (blue-colored lips or skin)
• Chest pain or pressure
• Difficulty gaining weight
• Fatigue
• Fainting or lightheadedness
• Irregular heartbeat or heart palpitations
• Shortness of breath
• Trouble feeding

Possible complications of aortic stenosis include:

• Aneurysm: Bulging or weakening of the aorta
• Dissection: Tearing of the aorta
• Heart failure: Heart doesn’t pump blood as it should
• Hypertrophy: Enlargement of the heart chambers
• Infective endocarditis: Infection affecting the lining of the heart, valves or blood vessels

Diagnosing Aortic Stenosis

The first sign of a potential heart problem in your child may be a heart murmur detected with a stethoscope during a routine exam. Your child’s primary care physician may refer them to a pediatric cardiologist at UH Rainbow for further testing and to confirm a diagnosis. Tests your child may undergo to diagnose aortic stenosis include:

Treatment for Aortic Stenosis in Children

If your child has mild or no symptoms, treatment may not be necessary. Your child may need to undergo periodic examination and testing by a pediatric cardiologist but otherwise their provider will simply keep an eye on them for any changes or worsening of symptoms.

For moderate or severe cases, treatment will depend on your child’s age, symptoms and other health-related factors. Your child’s pediatric cardiologist will work closely with your family to determine the best treatment option for your child. Valve procedures that may be used to treat aortic stenosis include:

• Balloon aortic valvuloplasty: This is a cardiac catheterization procedure in which your child’s doctor will insert a catheter with a deflated balloon into a blood vessel and guide it into the aortic valve. Once in place, the balloon is inflated to open the valve.
• Surgical aortic valvotomy: This is a surgical procedure to remove scar tissue from the aortic valve leaflets. This allows the leaflets to open properly in order to control blood flow.
• Aortic valve replacement: This is surgery to replace the aortic valve with either an artificial valve or one from a human donor organ or animal.
• Pulmonary autograft (Ross procedure): In this aortic valve replacement procedure, the surgeon removes the damaged aortic valve and replaces it with the child’s own pulmonary valve. The pulmonary valve and part of the pulmonary artery are then replaced using a donor organ. One of the benefits of this surgery is that it continues to work well as the child grows.

All procedures for aortic stenosis are performed in our state-of-the-art hybrid catheterization and surgical suites. Our facility offers cutting-edge technology, including the most advanced imaging available worldwide and 3-D capabilities. This helps our surgeons better understand and visualize each patient’s unique anatomy while making complex repairs, leading to improved patient safety and outcomes.

Follow-Up Care

Children who undergo valve repair or replacement for aortic stenosis can go on to live normal, healthy lives. Your child’s provider will give advice on ways to keep your child safe and healthy as they recovery from valve surgery. Even after successful treatment, a child with aortic stenosis will require periodic monitoring and follow-up by a pediatric cardiologist as well as continued cardiac care as they reach adulthood.