Double Outlet Right Ventricle

Double outlet right ventricle (DORV) is an abnormal heart issue in which your pulmonary artery and aorta connect to your right ventricle. In a typical heart, only one of them (pulmonary artery) connects there. This is a congenital heart condition, which means you’re born with it.

Usually, your pulmonary artery branches off from your right ventricle and your aorta branches off from your left ventricle. With DORV, your left ventricle connects to just a part of one of these arteries or no artery at all.

Double outlet right ventricle is a cyanotic heart disease. That means it can send blood without enough oxygen in it to your body. Also, your lungs may receive too much blood flow, which makes your heart work harder and can damage your heart and lungs.

DORV is rare. It happens in about 2 out of 10,000 newborns. Most will need surgery as infants.

Babies with a DORV heart defect often have other problems at birth, like:

• Ciliary problems: Problems develop with tiny, hairlike structures that line your baby’s airway.
• Coronary artery issues: An artery may start from the wrong place.
• Heterotaxy: Organs in your baby’s chest and belly aren’t in the usual places.
• Intestinal malrotation: A baby’s intestines twist in an unusual way.
• Pulmonary stenosis: The heart valve between a baby’s 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 this condition

Babies with double outlet right ventricle always have a ventricular septal defect (VSD), too. This hole in the septum, or wall between the two ventricles, lets blood flow between them.

Healthcare providers describe DORV types by the location of the VSD:

• DORV with subaortic VSD: The hole is just under the aorta.
• DORV with subpulmonary VSD: The hole is just under the pulmonary artery. Taussig-Bing anomaly is another name for this type.
• DORV with doubly committed VSD: There’s a hole under both major arteries.
• DORV with noncommitted VSD: The hole isn’t near the aorta or the pulmonary artery. Remote is another name for this type.

Symptoms of a DORV heart

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

• Blue or purple skin, lips or nails
• Fast breathing or shortness of breath
• Difficulty eating or gaining weight
• Heart murmur (extra sound in the heartbeat)
• A heart rate faster than 100 beats per minute
• Sweating a lot, like while feeding
• Unusual sleepiness or seeming “out of it”

DORV causes

Researchers don’t completely understand what causes double outlet right ventricle. It’s a problem with fetal development. In half of cases, it happens to babies who have genetic syndromes.

Complications of this condition

Possible complications of a DORV heart defect include:

• Abnormal heart rhythms
• Endocarditis
• Heart failure
• Heart valve issues
• Pulmonary hypertension

How doctors diagnose double outlet right ventricle

Sometimes, healthcare providers can find a heart defect like DORV 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 low oxygen levels, like blue lips. They’ll use a stethoscope to listen to your baby’s heart. Another test they’ll use is pulse oximetry screening. This is a painless way to measure the amount of oxygen in your baby’s blood.

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

• Noninvasive imaging tests: CTs, MRIs and X-rays take pictures from outside your baby’s body.
• Electrocardiogram (ECG or EKG): This painless test measures electrical activity in your baby’s heart.
• Echocardiogram: This pain-free, noninvasive 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 do you treat a double outlet right ventricle?

Almost all babies with DORV need open-heart surgery within their first year of life. Your healthcare provider will help you make decisions about a double outlet right ventricle repair. They’ll consider your child’s overall health, the type of DORV heart defect they have, and other issues with your baby’s heart or other organs.

Your baby’s surgeon may take one of these approaches to double outlet right ventricle treatment:

• 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.

When should my child see their healthcare provider?

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

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

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

Questions to ask your provider may include:

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

What can I expect if my child has a DORV heart?

Without surgery, a baby with double outlet right ventricle will develop high blood pressure in their lungs and heart failure.

With surgery, most babies who have this condition live to be adults. Anyone who’s had surgery for DORV needs lifelong care from a heart expert. Many children need another operation years later.

Because of the risk of infective endocarditis, some people with DORV need to take antibiotics before certain dental work. Taking care of your skin and teeth can also help prevent endocarditis.

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 life expectancy of a person with double outlet right ventricle?

With biventricular repair, people often live normal lives with at least an average lifespan. People who need other procedures may have shorter lifespans and need further surgery later in life. About 8 or 9 out of 10 people survive at least 10 years after surgery.