Double-Outlet Right Ventricle (DORV)
Information for teenagers and adults with double-outlet right ventricle
Double-outlet right ventricle (DORV) is a congenital heart condition in which both of the major arteries leaving the heart — the aorta and the pulmonary artery — arise predominantly from the right ventricle.
Normally, the pulmonary artery arises from the right ventricle and carries blood to the lungs, while the aorta arises from the left ventricle and carries oxygen-rich blood around the body.
In DORV, there is also almost always a ventricular septal defect (VSD) — a hole between the two pumping chambers of the heart. This VSD provides the pathway for blood from the left ventricle to leave the heart.
DORV describes a group of congenital heart abnormalities rather than one single condition. The anatomy and childhood operations can be very different from one person to another.
For this reason, understanding your individual anatomy and previous operations is particularly important if you have DORV.
How does DORV affect the circulation?
The effect of DORV depends mainly on:
Where the VSD is located
The relationship between the VSD and the aorta and pulmonary artery
Whether there is narrowing of the pathway to the lungs
The position and relationship of the major arteries
The size and function of the ventricles
Whether there are other associated congenital heart abnormalities
In some forms of DORV, the circulation before repair resembles Tetralogy of Fallot.
In others, it can behave more like transposition of the great arteries or a large VSD.
Some people have more complex anatomy involving the ventricles, heart valves or other structures.
This variation is why different people with DORV may have undergone very different operations.
Why is the VSD so important?
In DORV, the VSD is not simply a hole between the ventricles.
Because both major arteries arise predominantly from the right ventricle, the VSD often provides the main route for blood from the left ventricle to reach one of the great arteries.
The position of the VSD therefore has a major influence on the type of surgical repair that is possible.
For example, the VSD may be located close to:
The aorta
The pulmonary artery
Both major arteries
Neither major artery
Your congenital cardiologist can explain where your VSD was located and how your operation redirected blood through your heart.
How is DORV repaired?
There is no single operation for DORV.
The goal of surgery is generally to create efficient pathways so that oxygen-poor blood reaches the lungs and oxygen-rich blood reaches the body.
The operation chosen depends on the individual anatomy.
Repair using the VSD
In many people, surgeons create a tunnel or baffle inside the heart directing blood from the left ventricle through the VSD to the aorta.
The right ventricle then continues to pump blood towards the lungs.
Tetralogy of Fallot-type repair
When DORV is associated with pulmonary stenosis and the VSD is suitably positioned, the repair may resemble that used for Tetralogy of Fallot.
This can involve directing the left ventricle to the aorta and relieving obstruction between the right ventricle and pulmonary artery.
Rastelli-type repair
Some people require a Rastelli-type operation.
In this operation, an internal tunnel directs blood from the left ventricle through the VSD to the aorta.
A separate tube containing a valve — an RV–PA conduit — is then used to connect the right ventricle to the pulmonary arteries.
Arterial switch and other repairs
In some forms of DORV, particularly when the anatomy resembles transposition of the great arteries, an arterial switch operation or another complex repair may be performed.
Fontan circulation
In some people, the anatomy does not allow the heart to be separated into two effective pumping chambers.
These patients may instead follow a single-ventricle pathway, eventually resulting in a Fontan circulation.
If you have a Fontan circulation, many of the long-term considerations are different and are covered separately in our information about the Fontan circulation.
Why is it important to know what operation I had?
Two adults who were both born with DORV may have very different hearts after surgery.
Your long-term follow-up depends on the repair you actually have, not simply the original diagnosis of DORV.
If possible, it is useful to know:
The type of DORV you were born with
Where your VSD was located
Which ventricle now pumps blood to the aorta
Whether you have an internal tunnel or baffle
Whether you have an RV–PA conduit
Whether you have had an arterial switch
Whether you have a Fontan circulation
What previous heart operations and catheter procedures you have had
Your adult congenital heart disease team can often reconstruct this information from previous operation reports and cardiac imaging if you are unsure.
What problems can occur later in life?
The potential long-term issues depend on the original anatomy and type of repair.
Many people remain very well for many years after surgery.
Possible issues include the following.
Narrowing within the heart
An internal tunnel or baffle directing blood from the left ventricle to the aorta can sometimes become narrowed.
This may create left ventricular outflow tract obstruction, making it more difficult for the left ventricle to pump blood to the body.
Right ventricular outflow obstruction
Some people develop narrowing in the pathway from the right ventricle to the pulmonary arteries.
This may occur within the heart, at the pulmonary valve, in a previous surgical patch or within an RV–PA conduit.
Pulmonary regurgitation
People who have had a Tetralogy of Fallot-type repair may develop pulmonary regurgitation, where blood leaks backwards through the pulmonary valve.
Significant pulmonary regurgitation can gradually cause enlargement of the right ventricle.
RV–PA conduit problems
If your repair includes an RV–PA conduit, the conduit can gradually become narrowed or leaky.
Conduits do not last indefinitely, and many people require replacement during their lifetime.
In selected patients, it may be possible to implant a new pulmonary valve using a catheter procedure rather than open-heart surgery.
Ventricular dysfunction
Depending on the anatomy and previous operations, either ventricle can become enlarged or develop reduced function over time.
Regular imaging allows changes in ventricular function to be detected early.
Heart valve problems
Some people develop leakage or narrowing of one or more heart valves.
The valves requiring particular attention depend on your individual anatomy and previous repair.
Residual VSD
A small amount of blood flow may occasionally remain around the edge of a previous VSD patch or baffle.
Small residual defects may cause no significant problems but require monitoring.
Heart rhythm problems
People with repaired DORV can develop abnormal heart rhythms, particularly following complex or multiple heart operations.
These may include abnormal rhythms arising from the upper chambers of the heart or, less commonly, the ventricles.
Some people can also develop problems with the heart’s electrical conduction system and may require a pacemaker.
Tell your congenital heart team if you develop:
New palpitations
Unexplained dizziness
Fainting or near-fainting
A persistently unusually fast or slow heart rate
An unexplained deterioration in exercise capacity
Will I need more heart procedures?
Some people with repaired DORV never require another major operation.
Others require further procedures because conduits, valves, baffles or outflow pathways change over time.
Possible future procedures include:
Pulmonary valve replacement
RV–PA conduit replacement
Transcatheter pulmonary valve replacement
Ballooning or stenting of narrowed pathways or pulmonary arteries
Surgery for left or right ventricular outflow obstruction
Heart valve repair or replacement
Treatment of abnormal heart rhythms
Pacemaker or defibrillator implantation
The need for another procedure does not necessarily mean that the original operation has failed. Many childhood repairs are designed with the understanding that further treatment may eventually be required as the heart and patient grow.
How to stay well
Keep up with your congenital heart checks
People with DORV should have lifelong follow-up with an adult congenital heart disease specialist.
This remains important even when you feel completely well.
Your follow-up will be tailored to your particular anatomy and repair and may monitor:
Left and right ventricular size and function
Blood flow through surgical tunnels or baffles
Right and left ventricular outflow pathways
Pulmonary valve or RV–PA conduit function
Other heart valves
Pulmonary arteries
Residual VSDs
Heart rhythm
Exercise capacity
Stay active
Regular physical activity is encouraged for most people with repaired DORV.
The appropriate intensity depends on your ventricular function, heart valves, outflow pathways, heart rhythm and type of repair.
People with a good repair and normal heart function may be able to undertake a wide range of recreational exercise.
If you are considering competitive sport, high-intensity exercise or heavy resistance training, discuss this specifically with your congenital cardiologist.
Look after your teeth
Good dental hygiene and regular dental care are important.
Whether you require antibiotics before certain dental procedures depends on your individual repair.
People with some prosthetic valves or conduits, previous infective endocarditis or certain residual defects may require antibiotic prophylaxis.
Your congenital cardiologist or dentist can tell you whether this applies to you.
Don’t smoke or vape
Avoid smoking and vaping. Maintaining a healthy weight, blood pressure and cholesterol is also important for reducing additional cardiovascular stress.
Pregnancy
Many women with successfully repaired DORV can have a successful pregnancy.
However, because the anatomy and repairs vary so considerably, pregnancy risk needs to be assessed individually.
Risk may be higher when there is:
Reduced ventricular function
Significant valve disease
Significant obstruction within the heart or an RV–PA conduit
Pulmonary hypertension
Important abnormal heart rhythms
A Fontan circulation
Previous complex heart surgery
Women with DORV should have specialist pre-pregnancy assessment with an adult congenital heart disease and pregnancy team.
This allows the anatomy, ventricular function, heart valves, previous repair and medications to be reviewed before pregnancy.
Does DORV run in families?
Most people with DORV do not have another affected family member.
However, congenital heart disease can sometimes be associated with an underlying genetic condition, and people born with congenital heart disease have a higher chance of having a child with congenital heart disease than the general population.
Genetic counselling may be appropriate for some people.
A specialised ultrasound of the baby’s heart (fetal echocardiogram) is usually recommended during pregnancy.
Symptoms to tell your doctor about
Contact your doctor or congenital cardiology team if you develop:
New or increasing shortness of breath
A noticeable reduction in exercise capacity
Frequent or prolonged palpitations
Dizziness or near-fainting
Unexplained fainting
New swelling of your ankles or abdomen
Increasing fatigue
A significant change in your usual symptoms
Because problems such as conduit narrowing or pulmonary regurgitation can progress gradually, a persistent change in exercise capacity is worth discussing even if you otherwise feel well.
Emergency — get help immediately if
Seek urgent medical attention or call 000 if you develop:
Severe or rapidly worsening shortness of breath
Fainting during exercise
Fainting associated with palpitations
A sustained rapid heartbeat associated with dizziness or feeling very unwell
Severe chest pain
A sudden significant deterioration in your condition
Your regular check-ups
Follow-up for DORV may include:
Clinical review
Your cardiologist will ask about exercise capacity, breathlessness, palpitations and other symptoms and examine your heart and circulation.
ECG
An electrocardiogram records the electrical activity and rhythm of your heart.
Echocardiogram
An ultrasound can assess ventricular function, heart valves, outflow pathways and some aspects of previous surgical repairs.
Cardiac MRI
Cardiac MRI is particularly valuable in people with DORV. It can provide detailed information about the anatomy of the heart, previous surgical pathways, ventricular size and function, blood flow and pulmonary arteries.
Cardiac CT
CT may provide additional anatomical information, particularly about conduits, pulmonary arteries, coronary arteries and complex surgical pathways.
Exercise testing
Cardiopulmonary exercise testing can objectively assess your exercise capacity and help identify changes over time.
Heart rhythm monitoring
A Holter monitor or longer-term rhythm monitor may be recommended if you experience palpitations, dizziness or fainting.
Cardiac catheterisation
Catheterisation may be used to measure pressures and blood flow or to perform treatments such as stenting or transcatheter pulmonary valve replacement.
Questions for your doctor
What type of DORV was I born with?
What operation or operations have I had?
How is blood currently directed through my heart?
Do I have an internal baffle, conduit or artificial valve?
Are both of my ventricles functioning normally?
Is there any narrowing or significant valve leakage?
Am I likely to need another heart procedure in the future?
Do I have any restrictions on exercise, work, travel or pregnancy?
Do I need antibiotics before dental procedures?
How often should I have congenital heart follow-up?
This information is intended as a general guide for teenagers and adults with double-outlet right ventricle. DORV includes a wide range of anatomies and surgical repairs, so your congenital cardiologist can provide advice specific to the heart you were born with, the operations you have undergone and your current health.
