Double Outlet Right Ventricle

What is double outlet right ventricle?

Double outlet right ventricle (DORV) is an abnormal heart condition in which two major arteries (instead of one) connect to your right ventricle or heart chamber. This is a congenital heart condition, which means you’re born with it.

Usually, each of your major blood vessels or “great” arteries connects to one of your heart’s two ventricles.

• Your pulmonary artery branches off from your right ventricle and takes blood to your lungs to get oxygen.
• Your aorta branches off from your left ventricle and carries blood with fresh oxygen in it to the rest of your body.

With DORV, both great arteries connect to the right ventricle — either totally or in part. Your left ventricle has just a part of one of the great arteries, or no artery at all.

Double outlet right ventricle can cause your body to receive oxygen-poor blood instead of blood with plenty of oxygen in it. Also, your lungs may receive excessive blood flow, which makes your heart work harder and can damage your heart and lungs.

Babies with DORV often have other congenital (since birth) problems:

• Ciliary dysfunction: Problems develop with tiny, hairlike structures that line a baby’s airway.
• Heterotaxy: Organs in a baby’s chest and belly aren’t in the usual places.
• Intestinal malrotation: A baby’s intestines twist in an unusual way.
• Pulmonary stenosis: A baby’s pulmonary valve (between the right ventricle and pulmonary artery) is too small or stiff.
• Ventricular hypoplasia: One or both heart ventricles are smaller than they should be.

Types of double outlet right ventricle

Babies with DORV always have a ventricular septal defect (VSD), too. This hole in the septum or wall between the two ventricles lets blood flow through the hole and between the ventricles.

Healthcare providers classify DORV by the location of the hole:

• DORV with subaortic VSD: The VSD is just under the aorta.
• DORV with subpulmonary VSD (also called Taussig-Bing): The VSD is just under the pulmonary artery.
• DORV with doubly committed VSD: There is a VSD under both of the great arteries.
• DORV with noncommitted (or remote) VSD: The VSD isn’t near the aorta or the pulmonary artery.

How common is double outlet right ventricle?

DORV is rare. It happens just once for every 6,000 to 10,000 newborns.

What are the double outlet right ventricle symptoms?

Double outlet right ventricle symptoms usually appear during the first days or weeks after birth. They include:

• Cyanosis (blue or purple skin, lips or nails).
• Difficulty breathing, such as tachypnea (fast breathing) or shortness of breath.
• Difficulty eating or gaining weight.
• Heart murmur (extra sound in the heartbeat, which a healthcare provider can hear with a stethoscope).
• Tachycardia (heart rate faster than 100 beats a minute).
• Sweating a lot, such as while eating.
• Unusual sleepiness or seeming “out of it.”

What causes double outlet right ventricle?

Researchers don’t completely understand double outlet right ventricle causes. In 50% of cases, it happens to babies who have problems with their chromosomes, the cell structures that hold their DNA.

What are the complications of double outlet right ventricle?

Possible complications of DORV include:

Arrhythmias.

Heart valve issues.

Heart failure.

Pulmonary hypertension.

Endocarditis.

How is double outlet right ventricle diagnosed?

Sometimes, healthcare providers can find a heart defect before a baby is born. They may do this during a routine ultrasound screening called a fetal echocardiogram.

If not, a provider usually diagnoses double outlet right ventricle in the days or weeks after birth because of a baby’s symptoms.

During an exam, a provider will:

• Look for signs of cyanosis.
• Use a stethoscope to listen to your baby’s heart.
• Use pulse oximetry (pulse ox) screening to measure the amount of oxygen in your baby’s blood. The test is simple and pain-free.

What tests will be done to diagnose double outlet right ventricle?

Your baby’s healthcare provider may order one or more tests, like:

• Noninvasive imaging tests: Computed tomography (CT), magnetic resonance imaging (MRI) and X-ray take pictures from outside your baby’s body.
• Electrocardiogram (ECG or EKG): This painless test measures electrical activity in your baby’s heart.
• Echocardiogram (echo): This pain-free test measures sound waves from your baby’s chest to create images of their heart.
• Cardiac catheterization (or angiogram): For this test, a provider puts a thin tube through a vein and into your baby’s heart to get details on heart function.

How is double outlet right ventricle treated?

Almost all babies with DORV need open-heart surgery within their first year of life. Your healthcare provider will help you make decisions about surgery by considering:

• Other issues with your baby’s heart or other organs.
• Their overall health.
• The type of DORV they have.

Double outlet right ventricle surgery

Your baby’s surgeon may take one of the following approaches to double outlet right ventricle repair:

• Intraventricular repair: The surgeon creates a tunnel through the VSD to connect the aorta to the left ventricle.
• Biventricular repair or arterial switch: If both ventricles are in good shape and the DORV has a subpulmonary VSD (Taussig-Bing heart), the surgeon may recommend moving the aorta to the left ventricle.
• Univentricular repair: For more complex cases of DORV, the surgeon may recommend a Fontan operation. It redirects blood flow from your child’s lower body to their lungs.

Complications of the treatment

Most babies have good outcomes from surgery for DORV. But any heart surgery has risks, such as:

• Bleeding.
• Blood clots, which can cause a stroke or heart attack.
• Infection.
• Problems coming out of anesthesia.
• Respiratory failure.
• Unusual heart rhythms and heart block.

How long does it take to recover from double outlet right ventricle treatment?

Children who have a Fontan procedure may be in the hospital for a week or more. Recovery times vary by procedure and your child’s specific situation. Your surgeon will tell you what to expect.

Can double outlet right ventricle be prevented?

Because researchers are still trying to understand DORV’s cause, you can’t prevent it. You shouldn’t blame yourself, though. You didn’t make this happen.

What is the prognosis for double outlet right ventricle?

Without surgery, a baby with double outlet right ventricle will eventually develop:

• Cyanosis.
• Heart failure.
• Pulmonary hypertension (high blood pressure in the lungs).

With surgery, most babies who have double outlet right ventricle live to be adults. Anyone who’s had surgery for DORV needs lifelong care from a cardiologist or provider who specializes in taking care of your heart.

Can someone with a DORV repair have a child?

People who had a surgical repair for DORV can carry a pregnancy. But providers don’t recommend it in certain situations. If you’re an adult who had an operation for this condition as a child, talk to your provider if you’re considering pregnancy.

What is the double outlet right ventricle life expectancy?

With biventricular repair, people often live a normal life, with at least an average lifespan. People who need other procedures may have shorter lifespans and may need further surgery later in life. About 90% of people survive at least 10 years after surgery.

How do I take care of my child?

It’s important to take your child to their follow-up appointments to make sure they’re not developing any issues. Your healthcare provider will tell you how often your child will need checkups or more tests. Also, make sure your child is taking any medicines their provider prescribed.

Because of the risk of infective endocarditis, some people with double outlet right ventricle need to take antibiotics before certain dental procedures. Whether you take an antibiotic or not, taking care of your skin and teeth can help prevent endocarditis.

When should I see my healthcare provider?

Even after a surgical repair for double outlet right ventricle, a baby can have abnormal heart rhythms. Some people can develop heart failure years later. Contact your provider if your child has an abnormal heart rhythm or signs of heart failure, like chest pain or shortness of breath.

When should I go to the ER?

Take your child to the emergency room if they’re having trouble breathing and/or their nails, lips or skin have a blue tint.

What questions should I ask my doctor?

• What kind of DORV does my child have?
• What kind of surgery is needed?
• When should my baby have the surgery?
• What are the risks of having surgery?
• What are the risks of not having surgery?
• Will my baby need to see a specialist?
• Will the surgery fix the issue, or will my child need more surgeries?
• Will my baby need to take medications?