Health Library

Double Outlet Right Ventricle (DORV) in Children

In DORV, blood does not move through the heart the normal way because both arteries are connected to the right ventricle. Oxygen-rich and oxygen-poor blood can mix before leaving the heart. When this happens, less oxygen may reach the body.

DORV is considered a complex congenital heart defect. Many children with this condition also have other heart differences that affect how blood flows through the heart.

How the Heart Normally Circulates Blood

The heart has four chambers. The two upper chambers are called atria. The two lower chambers are called ventricles.

Blood moves through the heart in a steady cycle. First, oxygen-poor blood returns from the body to the right side of the heart. The right ventricle pumps this blood to the lungs through the pulmonary artery.

In the lungs, the blood receives oxygen. The oxygen-rich blood then returns to the left side of the heart.

Finally, the left ventricle pumps this oxygen-rich blood through the aorta to the rest of the body.

This system keeps oxygen-rich and oxygen-poor blood separate. That separation helps deliver enough oxygen to the body’s organs and tissues.

What Happens in Double Outlet Right Ventricle?

In DORV, both major arteries leave from the right ventricle instead of one artery coming from each ventricle.

All children with DORV also have a ventricular septal defect (VSD). A VSD is a hole in the wall between the two ventricles. This opening allows blood to pass between the chambers.

The position of the VSD affects how blood flows through the heart. DORV VSD types include:

• DORV with subaortic VSD: In this type, the VSD sits near the aorta. Blood from the left ventricle may move through the opening and flow into the aorta.
• DORV with subpulmonary VSD (Taussig-Bing anomaly): In this form, the VSD sits closer to the pulmonary artery. Blood may flow more easily toward the lungs.
• DORV with doubly committed VSD: In this type, the opening lies close to both arteries.
• DORV with noncommitted VSD: Here, the opening sits farther from both arteries. This can make blood flow patterns more complex.

Because each child’s heart anatomy is different, the way blood circulates can vary from child to child.

What Causes Double Outlet Right Ventricle?

DORV develops early in pregnancy while the baby’s heart is forming.

During normal development, the two main arteries—the aorta and pulmonary artery—separate and connect to the correct ventricles. In DORV, this process does not happen in the typical way.

Some children with DORV may also have genetic or chromosomal conditions, such as Trisomy 13, Trisomy 18, and 22q11.2 deletion syndrome. Others may have additional congenital heart defects.

Smoking, drinking alcohol, exposure to radiation and taking certain medicines during pregnancy can increase the risk of heart defects. Medicines linked to fetal heart defect risk include ACE inhibitors, certain antidepressants like paroxetine, lithium and specific anti-seizure drugs like valproate and carbamazepine.

In most cases, doctors do not know the exact cause.

What Are the Signs and Symptoms of Double Outlet Right Ventricle?

Double outlet right ventricle symptoms in children depend on how blood flows through the heart. Most babies show symptoms within days or weeks after birth. Others may develop signs later in infancy or childhood.

Common symptoms include:

• Bluish skin, lips or fingernails (called cyanosis)
• Fast breathing or breathing difficulty
• Trouble feeding
• Tiring easily
• Poor weight gain
• A heart murmur (whooshing sound) heard during an exam

Older children may also become tired more quickly during activity.

How Do Doctors Diagnose Double Outlet Right Ventricle?

A double outlet right ventricle diagnosis begins with a physical exam. Doctors may first suspect a heart defect after hearing a heart murmur. Imaging tests help confirm the diagnosis and show how the heart is structured.

Tests used for DORV diagnosis may include:

• Echocardiogram – a heart ultrasound that shows the chambers, valves and blood flow in the heart
• Electrocardiogram (EKG) – a test that measures the heart’s electrical activity
• Chest X-ray – an image of the heart and lungs
• Cardiac CT or MRI – detailed 3D images of the heart and blood vessels
• Cardiac catheterization – a procedure that measures pressure and blood flow in the heart using a thin tube placed into a blood vessel

Sometimes DORV is detected before birth during pregnancy through a fetal echocardiogram. 

What Are the Treatment Options for Double Outlet Right Ventricle?

Treatment depends on the child’s heart structure and overall health.

Most babies with DORV need surgery within the first few days or months to improve blood flow through the heart. Most newborns need medicines or oxygen to stabilize their condition before surgery.

Some babies will need a catheter-based intervention before they are able to have surgery. For example, some babies will need better blood flow to the lungs to allow them to grow bigger and improve their oxygen levels. This is done by interventional cardiologists, without surgery. The interventional cardiologist will place small flexible tubes called catheters into the blood vessels to reach the heart from the inside. For babies with DORV, they will place a stent into an artery called the Patent Ductus Arteriosus (PDA). Stents are small metal tubes that can be expanded to keep blood vessels open. The PDA is a blood vessel that is normally present as babies are developing and usually goes away after they are born. Your baby’s cardiologist will discuss with you if this is necessary for your baby.

Surgical treatment usually focuses on closing the ventricular septal defect and directing blood to the correct artery. Surgeons may also rebuild or adjust the heart’s outflow pathways. Open-heart surgery is done by opening the chest while the child is on a heart-lung machine. The machine takes over the work of the heart and lungs during surgery.

Some children need one surgery, while others need staged procedures performed over time. Doctors plan treatment based on each child’s unique heart anatomy.

Recovery and Follow-Up Care

After surgery, children stay in the hospital for up to 10 days while their heart begins to heal. During this time, doctors monitor heart function, oxygen levels and overall recovery. Full recovery usually takes two to three weeks at home.

Children continue to see a cardiologist for regular checkups. These visits often include imaging tests to monitor heart health.

Activity may be limited for a short time during recovery. Over time, many children return to normal daily activities.

Long-term heart care remains important for anyone born with a congenital heart defect.

What is the Long-Term Outlook for Children With Double Outlet Right Ventricle?

Many children with DORV do well after surgery. Long-term outlook depends on the type of DORV and whether other heart defects are present.

Children will need ongoing heart monitoring as they grow. Some may need additional procedures later in life.

With proper care, many children grow and develop normally. They may participate in school, activities and exercise with guidance from their cardiologist. As children reach adulthood, care may transition to doctors who specialize in adult congenital heart disease.

Living With Double Outlet Right Ventricle

Families play an important role in supporting their child’s heart health. Regular cardiology visits help doctors monitor heart function over time. Parents should also watch for symptoms such as breathing trouble, fatigue or changes in activity tolerance.

Encouraging safe activity, good nutrition and healthy growth can support a child’s development.

Living with a congenital heart condition can feel overwhelming at times. Many families find comfort and guidance through heart health resources and support groups.

Frequently Asked Questions

Is double outlet right ventricle life-threatening?

DORV can be serious, but many children do well with surgery and ongoing heart care.

Will my child need surgery?

Most children with DORV need surgery to improve blood flow through the heart.

Can DORV be detected before birth?

Yes. Doctors sometimes detect it during pregnancy with a fetal echocardiogram.

Can children with DORV play sports?

Many children can stay active. Activity recommendations depend on heart function, so families should follow guidance from their cardiologist.

How often will my child need heart checkups?

Children with DORV need regular heart checkups throughout life to monitor heart health.