Congenital Aortic Stenosis Surgery for Children: Ross Procedure

What is a Ross procedure for children?

A Ross procedure is a type of surgery. It fixes a birth defect in the heart that affects the aortic valve.

The heart has 4 chambers. The 2 lower chambers are the ventricles. The left ventricle pumps blood rich in oxygen to the body. It connects to the aorta, the main blood vessel leading to the body. Between the left ventricle and the aorta lies the aortic valve, one of the heart’s 4 valves. These valves help blood flow in the correct direction through the heart’s 4 chambers and out into the body. The pulmonary valve is the heart valve that lies between the right ventricle and the pulmonary artery. It sends blood that is low in oxygen out to the lungs.

The aortic valve normally has 3 small parts called leaflets. Sometimes only 1 or 2 leaflets develop instead of three. These leaflets may be abnormally thick and stiff. As a result, the valve may not be able to open as easily as it should when the heart squeezes. In this case, the left side of the heart has to work much harder to get blood out to the body. Over time, this can damage the heart muscle. It can lead to a condition called aortic stenosis. In aortic stenosis, the aortic valve does not open the right way because of an abnormal anatomy. This abnormality is often present at birth.

In a Ross procedure, a surgeon removes the abnormal aortic valve. The surgeon then replaces it with the child’s own pulmonary valve. The surgeon then uses a valve (conduit) from a donor who has died to replace the pulmonary valve.

Why might my child need a Ross procedure?

The Ross procedure is done to ease the symptoms of aortic stenosis. These are chest pain, extreme tiredness (fatigue), problems feeding or growing, or rapid or distressed breathing. Newborns with aortic valve problems are often very ill. The procedure decreases how hard the heart has to work. It also improves blood flow out to the body. Some children need treatment for aortic stenosis if they have too much pressure in the left ventricle, even if they don’t yet have symptoms. Aortic stenosis can damage the heart and cause symptoms later on.

The Ross procedure is not the only treatment for aortic stenosis. Balloon valvotomy is often a first treatment. It is a less invasive procedure that helps open up the valve. But it may not work permanently. It also may lead to a leaky valve. Your child’s healthcare provider may be more likely to advise the Ross procedure if your child has already had balloon valvotomy.

In some cases, it may be possible to fix the aortic valve or replace it with something other than the child’s own pulmonary valve. Each of these procedures has its own risks and benefits. One advantage of the Ross procedure is that the valve can grow with the child. The child will not outgrow it and need a future replacement. Your child’s healthcare provider may be more likely to advise a Ross procedure if your child has a certain kind of valve anatomy.

The Ross procedure is a technically demanding surgery. Not all surgical centers offer it. Ask your child’s healthcare provider about the pros and cons of the Ross procedure and other possible treatments.

In most cases, the cause of the aortic valve problem is not known. Sometimes aortic stenosis is present with other types of heart defects as well.

What are the risks of a Ross procedure for a child?

All procedures have risks. Some possible risks of this procedure include:

• Infection

• Excess bleeding

• Irregular heart rhythms

• Blood clots leading to stroke or heart attack

• Heart block (this can mean your child needs a pacemaker)

• Complications from anesthesia

Sometimes the surgery may not work. Another surgery may be needed. The new aortic valve also may become leaky with time, even if it works the right way at first. Or the newly placed pulmonary valve (conduit) may not grow with the child. It will need to be replaced in the future.

How can I help my child get ready for a Ross procedure?

Ask your child’s healthcare provider how to help your child get ready for a Ross procedure. Follow any directions your child is given for not eating or drinking before the day of the surgery. Your child may need to stop taking any medicines beforehand.

Your child may need some extra tests before the surgery. These might include:

• Chest X-ray. This shows the structures in and around the chest.

• Electrocardiogram. This checks the heart rhythm.

• Blood tests. These are done to check general health.

• Echocardiogram. This is done to see the heart and check blood flow through the heart.

• Heart catheterization. This is to better look at the coronary blood vessels or to measure the pressures in the heart and lungs.

The hair in the area of surgery may be removed. Your child may also get medicine to help them relax about 1 hour before the surgery.

What happens during a Ross procedure for a child?

Talk with your child’s healthcare provider about what to expect during the surgery. In general:

• Your child will get anesthesia before the surgery starts. Your child will sleep deeply and painlessly during the surgery. They won’t remember it later.

• The repair generally takes several hours.

• The surgeon will make a cut (incision) down the middle of your child’s chest. To reach the heart, the surgeon will separate the breastbone.

• Your child will be attached to a heart-lung machine during the surgery. This machine will act as your child’s heart and lungs during the procedure.

• The surgeon will remove the abnormal aortic valve.

• The surgeon will also remove the pulmonary valve. It will then be attached between the left ventricle and the aorta. This is where the abnormal aortic valve used to be.

• The surgeon will attach a donor pulmonary valve between the right ventricle and the pulmonary artery.

• Once all the repairs have been done and your child’s heart is beating sufficiently, the heart-lung machine will be removed.

• The breastbone will be put back together with wires.

• The surgeon will close the muscle and the skin. A dressing will be applied.

What happens after a Ross procedure for a child?

Ask your child’s healthcare provider what to expect. In general, afterward:

• Your child may be groggy and confused when they wake up.

• Your child’s vital signs will be closely watched. These include your child's heart rate, breathing, blood pressure, and oxygen levels.

• Your child will feel some soreness. But they should not feel severe pain. Pain medicines are available if needed.

• Your child may be able to drink as soon as the day after surgery. Your child can have regular foods as soon as they can handle them.

• Your child will likely need to stay in the hospital for 1 week or longer.

After you leave the hospital:

• Be sure to keep all follow-up appointments.

• Your child should be able to go back to normal activities soon. But they may be a little more tired for a while after the surgery.

• Ask your child’s healthcare provider about any exercise limitations. Children should not do any strenuous activities and physical activities that might include blows to the chest.

• Call your child’s healthcare provider if your child has a fever, more draining from the wound, or any new symptoms.

• Follow all the instructions your child’s healthcare provider gives you for medicine, exercise, diet, and wound care.

Most of the time, symptoms get better soon after surgery. Your child will need lifelong follow-up care with a cardiologist to watch for possible complications from the procedure. Your child may also need to take antibiotics before certain medical or dental procedures to prevent an infection of the heart valves.

Next steps

Before you agree to the test or the procedure for your child, make sure you know:

• The name of the test or procedure

• The reason your child is having the test or procedure

• What results to expect and what they mean

• The risks and benefits of the test or procedure

• When and where your child is to have the test or procedure

• Who will do the procedure and what that person’s qualifications are

• What would happen if your child did not have the test or procedure

• Any alternative tests or procedures to think about

• When and how you will get the results

• Who to call after the test or procedure if you have questions or your child has problems

• How much you will have to pay for the test or procedure