What is double outlet right ventricle in children?

Double outlet right ventricle (DORV) is a type of congenital heart malformation. This means it is present from birth. In this condition, the heart and the main blood vessels leaving the heart do not develop the right way. This can cause symptoms in your child.

A 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 (called the septum) between the ventricles and between the atria. It stops blood from flowing between the left and the right sides of the heart.

In a child with DORV, both the pulmonary artery and the aorta are connected to the same heart chamber, the right ventricle. In a normal heart, the aorta connects to the left ventricle, the ventricle that pumps blood to the body. The pulmonary artery normally connects to the right ventricle, the ventricle that pumps blood to the lungs. 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 opening is called a ventricular septal defect.

In many cases, DORV occurs with other heart malformations. For example, a heart valve may not work right. Or a ventricle may not be fully formed. DORV actually refers to a range of heart problems that share a common feature. With all of them, both great arteries are connected to the right ventricle. Depending on the exact nature of the DORV, the lungs might not get enough blood flow. Or they might get too much.

Only a small number of newborns have heart malformations. Of these, DORV is relatively rare.

What causes double outlet right ventricle in a child?

Researchers don't fully understand what causes DORV. Some substances (teratogens) can lead to a heart malformation if a mother is exposed to them during a vital time in her pregnancy. These might cause some cases of DORV.

Sometimes DORV results from 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.

Which children are at risk for double outlet right ventricle?

Most cases of DORV happen without any known cause. But they may be more likely to happen with certain syndromes. Some of these include:

• Trisomy 13 (Patau syndrome). This developmental disorder occurs when a child has 3 copies of chromosome 13 instead of 2.

• Trisomy 18 (Edwards syndrome). This condition slows growth and causes abnormalities in organ development. This happens because a child has 3 copies of chromosome 18 instead of 2.

• Robinow syndrome. This is a rare disorder that affects bone growth.

Each of these syndromes has its own symptoms, which sometimes include DORV.

What are the symptoms of double outlet right ventricle in a child?

Symptoms may vary depending on the exact heart abnormalities involved. Symptoms are often present at birth. But they may not appear until later. They can include:

• Bluish color (cyanosis) or pale skin

• Fast breathing or problems breathing

• Tiring easily, especially when feeding

• Failure to gain weight normally

• Swelling in the legs or belly (abdomen)

• Sleepiness or unresponsiveness

Surgery may greatly ease or stop these symptoms. That is even more likely if the surgery happens very early in life.

How is double outlet right ventricle diagnosed in a child?

Sometimes healthcare providers can diagnose DORV in a baby during pregnancy. A diagnosis after birth starts with a health history and physical exam. A cardiologist will need to do tests to make the diagnosis as well. The most important of these is an echocardiogram. This test shows the structure of the heart and the blood flow through the heart.

You may need other tests, too. These may include:

• Electrocardiogram (ECG). This is done to check the heart rhythm.

• Chest X-ray. This looks at heart size and the lungs.

• Cardiac MRI. This is done to look at the structure of the heart.

• Cardiac catheterization. This is done to get more information about the pressures in the different chambers of the heart and in the lungs.

• Blood tests. These tests look for oxygen levels in the blood or other factors.

How is double outlet right ventricle treated in a child?

DORV can be treated with surgery. Surgery can correct blood flow so that it moves the right way from the left ventricle to the aorta and from the right ventricle to the pulmonary artery. Surgery is also needed to fix the defect in the ventricular wall. And it can fix any other heart defects. The type of surgery will depend on the subtype and exact anatomy of the DORV and other heart problems. The timing of surgery varies. Healthcare providers may advise it soon after birth, within the first few months of life, or later. Some infants with DORV will need more than 1 surgery. The outcome depends on the type of DORV, other heart problems, and overall health at age of diagnosis.

Some children with DORV also need medicine. That may especially be the case if they haven’t yet had surgery and they have symptoms of heart failure. Treatment will vary based on the type of DORV and the severity of symptoms. Possible treatments include:

• Water pills (diuretics). These help to reduce swelling.

• ACE inhibitors or digoxin. These can improve heart output.

• Beta-blockers. These can reduce how hard the heart pumps.

Some children with DORV will also need blood thinners (anticoagulant medicine) to help prevent blood clots. These are only needed after certain kinds of surgery for DORV.

Most children with DORV go on to lead normal and active lives. But they will always need special follow-up care with cardiologists. Some may need follow-up surgery as adults.

What are possible complications of double outlet right ventricle in a child?

DORV can lead to a number of complications. The risk for complications varies according to the type of DORV, other heart conditions present, and the time of diagnosis. Early treatment can cut the chance of later problems. Possible complications include:

• Heart failure, which can lead to feeding and growth problems

• Very fast breathing or trouble breathing

• Harmful high blood pressure in the lungs

• Death

Children with heart abnormalities like DORV are also at higher risk for infection of the heart valves. To prevent it, your child’s healthcare provider might prescribe antibiotics before and after certain medical and dental procedures.

How can I help my child live with double outlet right ventricle?

Your child’s healthcare provider may give you more directions about helping your child manage DORV. But be sure to do the following:

• Ask the provider what kind of exercise is right for your child.

• Teach your child to eat a heart-healthy diet.

• Tell your child’s providers and dentists about your child’s DORV.

• Be sure your child sees a specialist in congenital heart disease regularly.

When should I call my child’s healthcare provider?

Call your child’s healthcare provider right away if your child has a hard time breathing or has other severe symptoms.

Key points about double outlet right ventricle in children

• Double outlet right ventricle is a type of heart malformation. It is present from birth.

• There are many subtypes of DORV that have different types of treatments and surgeries.

• This is a serious condition often treated early in life with surgery.

• DORV can cause serious complications. These include heart failure, high blood pressure in the lungs, and death.

• Many children with DORV can lead full and active lives. But they need lifelong follow-up care.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.

• Before your visit, write down questions you want answered.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.

• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.

• Ask if your child’s condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if your child does not take the medicine or have the test or procedure.

• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.