What is double outlet right ventricle surgery for children?

Double outlet right ventricle surgery is a procedure that fixes a type of heart malformation called double outlet right ventricle (DORV).

The normal heart has 4 chambers: 2 upper chambers (atria) and 2 lower chambers (ventricles). Blood flows from the right atrium into the right ventricle and from the left atrium into the left ventricle. There is a wall between the ventricles and between the atria. It stops blood from flowing between the left and right sides of the heart.

In a normal heart, the left ventricle is connected to the aorta and the right ventricle is connected to the pulmonary artery. In a child with DORV, both the pulmonary artery and the aorta are connected to the same heart chamber, the right ventricle. In DORV, these great arteries may be connected either partly or fully. There is also almost always an opening in the wall between the left and right ventricles. This hole is called a ventricular septal defect (VSD). The location of the VSD helps classify the different types of DORV. It also determines what kind of surgery needs to be done.

Much of the time, DORV happens with other heart malformations. For instance, a heart valve may not work right. Or a ventricle may not be fully formed. The type of surgery used to fix DORV varies based on the type of heart problems someone has. In most cases, the surgeon will fix the heart to establish the normal pattern of blood flow from the left ventricle to the aorta. They'll also make any other needed repairs. In some cases the DORV may be very complex, or 1 of the heart chambers may be very small. Then the surgeon may need to do a series of 3 surgeries to make the heart work with only 1 ventricle instead of 2 ventricles.

Why might my child need double outlet right ventricle surgery?

Surgery is needed for almost all cases of DORV. Children with DORV have trouble pumping blood through the body. That can lead to serious symptoms, such as breathing problems or not gaining weight. It can also cause serious complications. These include heart failure and high blood pressure in the vessels of the lungs. DORV surgery lets blood flow out to the body and lungs normally. Medicines can help with certain symptoms. But only surgery can fix the problem.

In some cases, healthcare providers might do surgery in the first few days or week after birth for an infant with severe symptoms. In other cases, the repair might happen later in the first year. Surgery is often needed even if symptoms are not at first severe. That’s because it may be harder to fix the problem later on. Over time, symptoms will happen in a child with uncorrected DORV.

The type of surgery your child needs for DORV may vary based on where the VSD is, and the size of the ventricles. It also depends on the other heart defects that are present. Your child’s healthcare provider will look at the heart in great detail before deciding on the type of surgery that will work best.

Sometimes DORV is caused by abnormalities in a child’s genes. Scientists have found many different genes that might lead to DORV. If a child has an abnormal number of copies of certain genes, that can lead to DORV. DORV is also linked to a number of genetic syndromes. But in most cases, the cause of DORV is unknown.

What are the risks of double outlet right ventricle surgery for a child?

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

• Excessive bleeding

• Infection

• Blood clots, which might lead to a stroke or heart attack

• Abnormal heart rhythms

• Complications from anesthesia

• Respiratory failure

• Damage to the phrenic nerve. This can cause paralysis of the diaphragm, which can cause breathing problems.

• Kidney failure

• Death

Risks will vary based on the kind of DORV present, other heart defects present, the type of surgery, and your child's overall health. Ask your child’s healthcare provider about the particular risks for your child.

How can I help my child get ready for double outlet right ventricle surgery?

Follow any directions your child is given for not eating or drinking before the surgery. Ask your child’s provider if your child needs to stop taking any medicine beforehand. Also ask the provider if there are other ways you need to prepare.

Your child may need to have some extra tests before the procedure. These might include:

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

• Electrocardiogram. This is done to check the heart rhythm.

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

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

• Cardiac MRI. This looks at the heart’s structure and function.

• Heart catheterization. This helps to learn more about the pressures in the chambers of the heart and the blood vessels connected to it.

What happens during double outlet right ventricle surgery for a child?

Talk with your child’s healthcare provider about what to expect during the surgery. The details of the surgery can vary a lot. They depend on the specific type of DORV and other heart problems present. In general:

• A healthcare provider will give your child anesthesia before the surgery starts. This is often through an IV (intravenous) line. Your child will sleep deeply and painlessly during the surgery. They won’t remember it later.

• The repair will take several hours.

• The surgeon will make an incision down the middle of the chest. To access the heart, the healthcare provider will separate the breastbone.

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

• In the simplest type of DORV repair, the surgeon will use a patch to make a tunnel between the left ventricle and the aorta.

• In other cases of DORV, the surgeon will do a procedure called an arterial switch. (This surgery is needed only if the arteries are reversed, and both are also attached to the right ventricle.) The surgeon will divide and surgically move the pulmonary artery and the aorta so that the right ventricle leads to the pulmonary artery and the left ventricle empties into the aorta. The surgeon will also switch the coronary arteries.

• Very complicated or severe cases of DORV may call for 3 separate surgeries. These surgeries will result in a functioning heart that uses only 1 ventricle.

• The surgeon will fix any other abnormalities, such as a faulty valve.

• Once all the repairs have been done and the heart is beating normally, the heart-lung machine will be removed.

• The breastbone will be sewn together with small wires.

• The muscle and the skin incisions will be closed. A bandage will be applied.

What happens after double outlet right ventricle surgery for a child?

Talk with your child’s healthcare provider about what will happen after the surgery. Afterward, you can generally expect the following:

• Your child will spend several hours in a recovery room. Or the surgery team may take your child straight to the intensive care unit (ICU).

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

• Your child will receive pain medicine if needed.

• Your child’s healthcare provider may order follow-up tests, like an electrocardiogram or an echocardiogram.

• Your child will likely be able to go home 2 to 3 weeks after surgery. Your child may need to spend a longer time if there were surgical complications, or if your child was in critical condition before the surgery.

At home after the procedure:

• Ask what medicines your child needs to take. Your child may need to take certain medicines for a short time after the surgery, such as blood thinners or antibiotics. Give pain medicines as needed.

• Keep all follow-up appointments.

• Call your child’s healthcare provider if your child has more swelling, more bleeding or drainage, a fever, or severe symptoms. A little drainage from the site is normal.

• Follow all the instructions your child’s provider gives you about medicine, activity, diet, and wound care.

• For a while after the procedure, your child might also need to have antibiotics before certain medical and dental procedures. This medicine can help prevent an infection of the heart valves.

Your child will need close follow-up care with a heart specialist after the surgery. People born with DORV will need to see such a specialist from time to time for the rest of their lives. Most children go on to lead full and active lives as adults.

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