Conditions & Treatments
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Double outlet right ventricle (DORV)

Disease Information

In-Depth

The DORV defect is challenging to treat. But the dedicated, compassionate team at Children’s Hospital Boston is well-qualified to care for your child.

We’re known for our science-driven approach. In fact, we’re home to the world's most extensive pediatric hospital research enterprise; and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your child is precious, and not just a patient.

What are the different types of DORV defect?

There are four major types of DORV defect, which can be classified by the location of the hole (ventricular septal defect or VSD) with respect to the aorta and the pulmonary artery. The most common of these types is a DORV where the VSD is located:

just below the aorta (subaortic)

The location of the VSD also defines the other three types of DORV. These other types locate the VSD:

below the pulmonary artery (subpulmonary)
below both the aorta and the pulmonary artery (doubly committed)
in a position away from the great arteries (non-committed)

These four types of DORV—as well as the presence or absence of associated pulmonary stenosis and other defects—will determine how severe your baby’s condition and symptoms are.

Additional but somewhat less common defects that may be present with DORV include:

What causes the DORV defect?

It’s important for parents to understand that you’ve done nothing to cause DORV and its accompanying defects. Nothing you've ingested or have been exposed to environmentally has been definitively linked to having a child with DORV.

Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. Familial cases have been reported, but no genetic link has been confirmed. Occasionally, chromosomal abnormalities have been found in some children with DORV.

What are the symptoms of DORV?

Your child’s type of DORV and related defects will determine her symptoms, the severity of these symptoms and the age at which they first occur. While each child might experience symptoms differently, typical symptoms are:

In infants and babies:

rapid breathing
rapid heartbeat
sweating
disinterest in feeding or tiring while feeding
poor weight gain
blue color of the skin, lips and nailbeds (cyanosis)
heart murmur (detected by doctor)

In older children:

fatigue
shortness of breath

If your child’s pediatrician notices any of these symptoms, the doctor may refer you to a pediatric cardiologist for testing, diagnosis and a determination of treatment.

How do you treat DORV?

The DORV defect is always treated with one or more operations performed in the baby’s first few years. The goals of these surgeries are to:

make it possible for blood to flow from the left ventricle only to the aorta,
and from the right ventricle only to the pulmonary artery
correct other defects that may be present

Learn more about Treatment & care for DORV.

Will my child be OK?

Open heart surgery at Children’s has among the highest success rates in the United States among large pediatric cardiac centers. With our advanced surgical techniques and timely, family-centered care, the overwhelming odds are that your child will have a successful surgical DORV repair.

Note: Infants who’ve had initial surgery for a single ventricle defect are typically enrolled in Children’s Home Monitoring Program between the baby’s Stage I and Stage II operations in the Fontan sequence. For more, see “Children’s Home Monitoring Program for your child” in Treatment & care.

What about later in life?

We've found that the large majority of DORV defects can be corrected. Abnormal heart rhythms and other long-term complications can occur—particularly if patients undergo repair at a later age—but these are usually manageable.

Your cardiologist will help you create a long-term care program as your baby grows into childhood, the teen years and even adulthood. Most people who've had congenital heart disease repair will have an ongoing relationship with their cardiologist; we will consult and treat your child if complications arise, and will advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

Where can my child find care and support when she grows up?

Our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.

BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.

The Adult Congenital Heart Association (ACHA) is a national not-for-profit organization dedicated to improving and extending the life of adults with congenital heart defects. The organization serves and supports the many adults with congenital heart defects (now thought to number at least 1 million), their families and the medical community, as well as conducting research and providing advocacy, outreach and education.

FAQ

Q: What is double outlet right ventricle (DORV)?

A: In the congenital heart defect double outlet right ventricle defect (DORV), the pulmonary artery and the aorta—the heart’s two great arteries—both arise from the right ventricle. Most DORV defects are accompanied by a ventricular septal defect (VSD), which is a hole in the tissue wall (septum) that normally separates the right and left ventricles. Your child may also have additional heart defects associated with DORV.

click to enlarge

Q: If my child has DORV, will she be OK?

A: Open heart surgeries at Children’s Hospital Boston have among the highest success rates in the United States among large pediatric cardiac centers. With our advanced surgical techniques and timely, family-centered care, in most cases the overwhelming odds are that your child will have a successful surgical DORV repair.

Q: How does Children’s treat DORV?

A: The skilled cardiac surgeons at Children’s treat DORV with a series of surgical repairs performed in the baby’s first few years. The goals of these surgeries are to:

make it possible for blood to flow from the left ventricle only to the aorta,
and from the right ventricle only to the pulmonary artery
correct other defects that may be present

Learn more about Treatment & care for DORV.

Q: What are the symptoms of DORV?

A: Typical symptoms in infants and babies can include:

rapid breathing and/or heartbeat
sweating
blue color of the skin, lips and nailbeds (cyanosis)
disinterest in feeding or tiring while feeding
poor weight gain
heart murmur (detected by doctor)

In older children, symptoms can include:

fatigue
shortness of breath

If your child has any of these symptoms, your pediatrician may refer you to a pediatric cardiologist for testing, diagnosis and a determination of treatment.

Q: If my child has DORV, what should I ask my Children’s doctor?

A: If your child is diagnosed with double outlet right ventricle, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you. Lots of parents find it helpful to jot down questions as they arise—that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

Some of the questions you may want to ask include:

What further tests will you perform to diagnose my child?
What actions might you take after you reach a diagnosis?
Are there alternative therapies?
Will my child be OK if she has congenital heart disease?
Will there be restrictions on my child’s activities?
Will there be long-term effects?
What can we do at home?

Q: When is DORV usually diagnosed, and how?

A: DORV can be picked up on a screening ultrasound done on your fetus, but it's usually diagnosed at or shortly after birth, particularly if a newborn is born with a bluish tint to her skin. (Occasionally, symptoms show up in a young child.)

Your Children’s cardiologist will detect a heart murmur, measure blood oxygen levels (non-invasively) and order diagnostic testing, which could include EKG, cardiac ultrasound, cardiac MRI or chest x-ray.

Q: What should we do at home after DORV surgery?

A: Consult your child’s cardiologist for guidance on follow-up care—including:

a nutritional program to encourage weight gain
an oral hygiene program to prevent infection, and
an appropriate exercise regimen to build body mass and achieve fitness

As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.

Q: What is the long-term outlook for DORV-affected children?

A: The large majority of DORV defects can be corrected, so the outlook for your child is good. Arrhythmias and other long-term complications can occur—particularly if patients undergo repair at a later age—but these are usually manageable.

Most people who've had congenital heart disease repair have an ongoing relationship with their cardiologist; we will treat your child if complications arise, and will advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

Q: What causes DORV?

A: It’s important for parents to understand that you’ve done nothing to cause DORV and its accompanying defects. Nothing you have ingested or have been exposed to environmentally has been definitively linked to having a child with DORV. Occasionally, chromosomal abnormalities have been found in some children with DORV.

Q: What is Children’s’ experience treating congenital heart defects?

A: Children’s surgeons treat some of the most complex pediatric heart conditions in the world, with success rates approaching 98 percent—among the highest in the nation among large pediatric cardiac centers. Our experts have pioneered some of medical science’s most advanced heart treatments, now in use around the globe. Children’s is the only major pediatric heart center in the world performing fetal heart interventions for certain congenital defects.

Q: What heart research and innovations are coming from Children’s?

A: A significant amount of the groundbreaking cardiac research being conducted at Children’s Hospital Boston aims to refine and advance the open heart surgery and catheterization procedures that correct congenital heart defects in newborns and young children—including double outlet right ventricle.

Read more about Children’s cardiac research initiatives and projects in cardiology research.

Causes

It’s important to understand that as parents, you’ve done nothing to cause DORV and its accompanying defects. Nothing you've ingested or have been exposed to environmentally has been linked to having a child with DORV. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.

Symptoms

In infants and babies:

rapid breathing
rapid heartbeat
sweating
disinterest in feeding or tiring while feeding
poor weight gain
blue color of the skin, lips and nailbeds (cyanosis)
heart murmur (detected by doctor)

In older children:

fatigue
shortness of breath

When to seek medical advice

Call your health care provider immediately if your baby or child is having difficulty breathing or is breathing rapidly, has a bluish color, tires easily or is uninterested in eating.

Questions to ask your doctor

If your child is diagnosed with double outlet right ventricle, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you. Lots of parents find it helpful to jot down questions as they arise—that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

Some of the questions you may want to ask include:

What is happening to my child, and why?
What further tests will you perform to diagnose my child?
What actions might you take after you reach a diagnosis?
Are there alternative therapies?
Will my child be OK if she has congenital heart disease?
Will there be restrictions on my child’s activities?
Will there be long-term effects?
What can we do at home?

Who’s at risk

Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. So, it’s difficult to predict who’s at risk. Familial cases have been reported. Occasionally, chromosomal abnormalities have been found in some children with DORV.

Complications

Children who've undergone DORV repair could be susceptible to congestive heart failure, or lung damage resulting from high blood pressure in the lungs. However, with improved treatment techniques and follow-up care, these complications are becoming increasingly less common.

There’s a risk of arrhythmias occurring, especially for patients who've had their repairs later in life. And some people who've undergone DORV repair will require lifelong medication to prevent heart infections.

Long-term outlook

The large majority of DORV defects can be corrected, so the outlook for your child is good. Arrhythmias and other long-term complications can occur—particularly if patients undergo repair at a later age—but these are usually manageable.

Your cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and even adulthood. Most people who have had congenital heart disease repair have an ongoing relationship with their cardiologist; we will consult and treat your child if complications arise, and will advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.

For teens

If you’re a teen with a congenital heart defect, you have a lot to cope with. Besides the typical issues any teenager faces—from social acceptance to body changes and more—you’ll also have to deal with medical appointments and procedures … some delay of your natural wish for independence … feeling different … and assuming a lot of personal responsibility for maintaining your own good health.

If you feel overwhelmed, depressed or anxious through this important time in your transition to adulthood, speak to your doctor or counselor to get help.

For adults

If you were treated for congenital heart disease as a child, you’re probably being followed by your cardiologist, since complications from early heart disease can arise in adulthood.

You may need lifelong monitoring and medication, since you’ll continue to be at some risk for arrhythmias, blood clots, infections, heart failure or stroke. Going forward, your cardiologist will also advise you on activity levels, pregnancy issues and certain lifestyle choices.

Fortunately, Children’s can help adults with congenital heart defects. Many adults who were patients as babies or children continue to be monitored by the clinicians who have followed them since childhood.

In addition, our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.

BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.

What you can do at home

Your child’s cardiologist will offer recommendations for post-operative follow-up care, including:

a nutritional program to encourage weight gain
an oral hygiene program to prevent infection, and
an appropriate exercise regimen to build body mass and achieve fitness

As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.

Prevention

It’s important to understand that as parents, you’ve done nothing to cause DORV and its accompanying defects, and there are no precautions you could have taken to prevent it. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.

DORV Glossary

aorta: one of the heart’s two great arteries. In a normal heart, the aorta arises from the left ventricle and carries oxygen-rich blood out to the body.
arterial switch procedure: An operation necessary only if, in addition to the arteries arising from the right ventricle, they are also reversed in relation to each other (transposition of the great arteries [TGA]). In TGA, the aorta and pulmonary artery are divided and “switched” so that the blue blood goes out the pulmonary artery and the red blood goes out the aorta.
cardiac catheterization: an invasive diagnostic procedure performed under sedation to obtain detailed visual information and measurements about the structures inside the baby’s heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta.
cardiac/cardio-: pertaining to the heart
cardiac magnetic resonance imaging (MRI): a non-invasive diagnostic tool using 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child's heart
cardiac surgery: surgical procedure performed on the heart or one of the major arteries or veins connected to the heart.
cardiac surgeon: doctor who performs surgery on the heart. A pediatric cardiac surgeon performs surgery on the hearts of infants and children.
cardiologist: doctor who diagnoses and treats heart problems non-surgically. A pediatric cardiologist treats infants, children and some adults with heart problems.
The Center for Families at Children’s: dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care
chest x-ray: a diagnostic tool chest to evaluate the size and spatial relationships of the heart within the child’s chest, as well as the presence of defects associated with DORV. It may also be used to see if there is too little or too much blood going to the lungs.
coarctation of the aorta: a less common defect associated with DORV in which there is a narrowing of the aorta, the main artery that delivers oxygen-rich (red) blood to the body.
cyanosis (cyanotic): blue color of skin, lips and nailbeds caused by a reduction in the amount of oxygen-rich (red) blood circulating in baby’s bloodstream
congenital heart defect: heart defect present at birth. The heart usually starts to form in the first eight weeks of fetal development. It is thought that most congenital heart defects develop during this period.
diagnosis: medical determination of illness or disease based on history, physical examinations and advanced technology diagnostic testing tools
double outlet right ventricle (DORV): congenital (present at birth) heart defect in which the pulmonary artery and the aorta—the heart’s two great arteries—both arise from the right ventricle.
echocardiogram (echo, cardiac ultrasound): a diagnostic tool that evaluates the structure and function of the heart using sound waves that produce a moving picture of your child’s heart and heart valves. The ultrasound can be used to understand flow in the different chambers and to estimate pressures.
electrocardiogram (ECG, EKG): a diagnostic tool that evaluates the electrical activity of your child’s heart. An EKG is usually the initial test for evaluating the causes of symptoms and detecting heart abnormalities, including DORV.
Fontan sequence: a series of two or three reconstructive operations performed only on children whose DORV defect results in just one full-sized functioning ventricle (a type of single ventricle defect). The Fontan operations are usually performed at intervals starting within days or months after birth and ending at a few years of age.
intraventricular repair: the most typical surgical procedure for DORV. Surgeons create a kind of tunnel (“baffle”) connecting the left ventricle to the aorta. A patch placed within the right ventricle directs left ventricle flow to the aorta.
mitral valve abnormality: a less common defect associated with DORV. If the valve between the left atrium and the left ventricle (mitral valve) is abnormally formed, it can impede flow or allow backward flow of blood from the left ventricle into the left atrium.
modified Blalock-Taussig shunt: a surgical procedure to control blood flow to the lungs. If pulmonary (valve) stenosis (PVS, PS) is present, causing an insufficient amount of blood flow to the lungs, a shunt (tube) may be inserted between the aorta and pulmonary artery to increase blood flow, although in DORV a reparative operation is usually performed.
pulmonary artery: one of the heart’s two great arteries, which normally arises from the right ventricle and carries oxygen-poor blood to the lungs, where it receives oxygen.
pulmonary artery banding: One of the complications associated with DORV is too much blood flow to lungs—the opposite problem from pulmonary valve stenosis. With pulmonary artery banding, a band is secured around the pulmonary artery to narrow it and thereby limit blood flow to the lungs.
pulmonary valve stenosis (PVS, PS): a congenital (present at birth) heart defect in which the pulmonary valve is unable to open completely, making it harder for blood to flow from the right ventricle to the lungs
right aortic arch: when the aorta arches to the right instead of to the left of the trachea (main breathing tube)
shunt: an artificial connection of blood vessels in order to redirect additional blood to the lungs when there is pulmonary stenosis or pulmonary atresia (complete blockage)
single ventricle defect (SVD): a congenital (present at birth) heart defect in which the heart has only one fully functioning ventricle
sporadic: occurring by chance, occasionally, not inherited
symptoms: the presenting reasons why a child needs medical attention. A symptom’s characteristics—including but not limited to onset, quality, triggers and severity—help diagnosticians to determine an illness or disease, or to determine what further testing is needed to determine the illness or disease.
transposition of the great arteries (TGA): a defect occasionally associated with DORV in which, in addition to the arteries arising from the right ventricle, they are also reversed in relation to each other. An arterial switch operation may be performed to correct this defect.
ventricular septal defect (VSD): a congenital (present at birth) heart defect in which there is an opening in the ventricular septum (dividing wall) between the two lower chambers of the heart (right and left ventricles)