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Double Outlet Right Ventricle

It can be a big shock to learn your baby has a serious heart problem.

You probably have all sorts of worries and questions when you think about your new baby. But finding out their arteries aren’t attached to their heart like they should can take that worry to the next level. The good news? Double outlet right ventricle (DORV), is a congenital (born with it) condition that can be repaired early with surgery — usually within your child’s first year.

At Cleveland Clinic Children’s, our expert pediatric cardiologists and pediatric surgeons treat kids with all kinds of heart conditions, including DORV. We’ll create a personalized treatment plan for your child and support your entire family throughout the process. We know that managing and coping with your baby’s heart condition can be stressful, so we’ll be sure to explain everything you need to know all along the way.

Why Choose Cleveland Clinic Children’s for Double Outlet Right Ventricle Care?

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Patient-centered care:

We treat children with DORV from birth, or even before birth. And we provide a lifetime of follow-up care and specialized treatment that’s personalized to your child’s needs and health.

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Recognized expertise:

Our providers participate in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a group of more than 60 U.S. cardiac centers. The NPC-QIC creates recommendations to help increase life quality and survival rates for children with these types of heart conditions.

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Skilled collaborative providers:

In addition to our expert pediatric cardiologists and heart surgeons, your child will see providers from different specialties at Cleveland Clinic Children’s if they have other health challenges linked to DORV. From brain development to lung problems, we’re here to make sure your baby gets the care and treatment they need. Meet our team.

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Convenient care:

Before you take your baby home, we’ll make sure your family is well-prepared. We’ll give you a monitoring system so you can keep track of how your baby is doing. And we’re always here to answer any questions along the way.

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Personalized care:

We’ll customize your child’s treatment to their specific needs — keeping a close eye on any changes to how their heart is working. We’ll also talk about any other health concerns you’ve noticed.

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National recognition:

Cleveland Clinic Children’s is a trusted healthcare leader. We’re recognized throughout the U.S. for our expertise and care.

Diagnosing Double Outlet Right Ventricle at Cleveland Clinic Children’s

Finding and treating your baby’s double outlet right ventricle as early as possible helps your child thrive as they grow. Sometimes, DORV can be seen on a prenatal ultrasound. Other times, we’ll discover it in the days or weeks after birth. Symptoms like blueish skin (cyanosis), breathing problems, a heart murmur or a fast heart rate can be red flags.

To diagnose double outlet right ventricle, we start by listening to your child’s heart with a stethoscope. We also measure the amount of oxygen in their blood using a tool called a pulse oximeter (pulse ox).

Babies with DORV also have a hole (ventricular septal defect, or VSD) in the wall between the bottom chambers of their heart (right and left ventricles). They often have other heart conditions, too. If we suspect DORV or any other heart conditions, we may do several tests.

Echocardiogram (Echo)

This painless ultrasound helps us take a better look inside your child’s heart. We’ll place a hand-held wand on their chest and move it around to see different areas of their heart.

Electrocardiogram (EKG)

An EKG is also painless and records electrical activity in your baby’s heart. We’ll place pads connected to wires (electrodes) on their chest, arms and legs and read their heart signals on a computer monitor.

Cardiac catheterization

Sometimes, we’ll do a cardiac catheterization to get more information about your baby’s heart before doing surgery. Your child’s provider will insert a thin tube (catheter) through a blood vessel in your baby’s arm or leg. Then they’ll inject a dye (contrast material) through the catheter, which moves into their heart chambers. By watching the contrast material move through your child’s heart, your child’s provider can see how it’s working. Your baby will be asleep during this procedure and not feel any pain.


Our healthcare providers see patients at convenient locations throughout Northeast Ohio.

Treating Double Outlet Right Ventricle at Cleveland Clinic Children’s

DORV is different for each child. That’s why your baby’s personalized care plan is based on their specific condition and how bad it is. Their treatment will also depend on whether they have other heart conditions.

Most children with DORV need surgery. If your child is diagnosed with DORV before or at birth, we may suggest one of several kinds of surgery. We know that the thought of your baby having heart surgery can be overwhelming. But please know that our pediatric heart surgeons are highly skilled and extremely experienced. And they’ll walk you through what to expect before your baby ever enters the operating room (OR).

Intraventricular baffle repair

Our pediatric surgeon will create a tunnel that connects your child’s left ventricle to their aorta. They’ll place a patch inside your child’s heart to direct blood flow between their left ventricle and their aorta.

Biventricular repair (arterial switch)

During this surgery, we’ll move your child’s aorta from their right ventricle to their left ventricle. We’ll also close the hole between their right and left ventricles.

Blalock-Taussig (BT) shunt

Our pediatric surgeon will insert a soft tube (shunt) between your child’s aorta and their pulmonary artery. This shunt helps to direct blood from their left ventricle to their lungs. We’ll remove this shunt when we repair their heart disorder during a later surgery. This procedure can be done without open-heart surgery.

Glenn procedure

We often do this surgery when your baby is 4 to 6 months old to improve blood flow to their lungs. The Glenn procedure is an open-heart surgery, so we’ll need to open your child’s chest and heart to do it. Our pediatric surgeon will disconnect your child’s superior vena cava and attach it to their pulmonary artery. This lets blood from your baby’s upper body flow directly to their pulmonary artery and to their lungs.

We’ll provide dedicated care and support throughout the process. We want to ease your fears and put your child on the path to recovery with the best possible outcomes.

Univentricular repair (Fontan procedure)

When your child is 24 to 48 months old, they might need a type of open-heart surgery called a Fontan procedure. Our pediatric surgeon will detach your child’s inferior vena cava (large vein that carries blood from the lower half of your child’s body to their heart) and connect it to their pulmonary artery using a tube. The Fontan procedure lets blood returning from the lower part of your child’s body flow directly to their pulmonary artery and then lungs instead of going through their heart first.

Recovery After Heart Surgery

Your baby will usually need to spend at least one to two weeks in the hospital after heart surgery. During this time, they’ll have supervised 24-hour care. And we’ll help you learn how to best care for your child when it’s time for them to go home. We’re here to help your baby succeed and thrive as they heal and grow.

Follow-up Care

After treatment ends, our pediatric cardiologists will continue to see your child for regular follow-up exams. They’ll keep an eye on things and check for any leaking or narrowing in your child’s heart valves. And when the time is right, we’ll introduce them to our adult cardiology team. We’re here for your child with compassionate and seamless care for the rest of their life.

Taking the Next Step

Heart problems can be stressful and overwhelming — especially if your baby has one. But getting an early diagnosis and treatment for double outlet right ventricle can prevent their condition from getting worse. At Cleveland Clinic Children’s, we’re here to help make that happen with skilled, personalized, compassionate care. We want your child to have a healthy recovery and go on to enjoy a full, active life.

Getting an appointment with Cleveland Clinic Children’s double outlet right ventricle experts is easy. We’re here to help your baby.


Getting an appointment with Cleveland Clinic Children’s double outlet right ventricle experts is easy. We’re here to help your baby.

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