Double Outlet Right Ventricle: Causes, Symptoms, and Treatment
Medically reviewed by Achuta Uppu, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Double Outlet Right Ventricle?
A pediatric cardiologist, a heart doctor for children, usually leads care for double outlet right ventricle. A heart surgeon repairs the defect. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is double outlet right ventricle?
Double outlet right ventricle (DORV) is a heart disease that is present from birth. Doctors call this a congenital heart defect. A ventricle is a pumping chamber of the heart. The aorta is the main artery that carries oxygen-rich blood to the body. The pulmonary artery carries oxygen-poor blood to the lungs.
In DORV, both of these arteries come from the same pumping chamber, the right ventricle. A second defect, a hole between the two ventricles called a ventricular septal defect (VSD), always occurs with DORV. Oxygen-rich blood flows through the VSD into the right ventricle. This mixing helps the baby. Even so, the body may not get enough oxygen, and the heart works harder. There are several types of DORV. They differ by where the VSD sits compared with the two arteries. DORV is reported in about three to nine in 100,000 live births.
Symptoms
The symptoms and how serious they are depend on the type of DORV. A narrowed pulmonary valve also affects the condition. Symptoms may include:
- Poor feeding because the baby tires easily.
- Bluish color of the skin and lips, called cyanosis.
- Poor weight gain, called faltering weight.
- Pale color and sweating.
- Swollen legs or belly.
- Trouble breathing.
- Clubbing, which is thickening of the nail beds of the toes and fingers. This is a late sign.
Doctors may find an enlarged heart, a heart murmur (an extra sound in the heartbeat), fast breathing, and a fast heartbeat.
Causes and who gets it
People with DORV often have other heart defects. Examples are a narrowed aorta (coarctation of the aorta), problems with the mitral valve, and a narrowed pulmonary valve. Another example is pulmonary atresia, where the pulmonary valve does not form properly.
DORV can happen on its own or with other heart and body defects. More than 40% of people with DORV have chromosome changes. Most of those people also have defects outside the heart.
How doctors diagnose it
Tests to diagnose DORV include:
- Chest x-rays.
- Echocardiogram, an ultrasound exam of the heart.
- Cardiac catheterization, which passes a thin, flexible tube into the heart to measure blood pressure and inject dye for special pictures.
- Heart MRI.
DORV can also be found before birth. A study found that it can be diagnosed prenatally with impressive accuracy.
Treatment
Treatment requires surgery. The surgeon closes the hole in the heart and directs blood from the left ventricle into the aorta. Surgery may also be needed to move the pulmonary artery or the aorta.
The type and number of operations depend on the type of DORV, how severe it is, other heart problems, and your child's overall condition. Newborns with symptoms might need a first procedure before the full repair. Babies without symptoms could have an elective repair at 3 to 6 months of age.
Children with this heart condition may need antibiotics before surgeries or dental treatments. This prevents infections in the heart. Follow your doctor's instructions about this.
How well a baby does depends on the size and location of the VSD, the size of the pumping chambers, and other problems such as coarctation of the aorta. Complications can include heart failure and high blood pressure in the lungs. Untreated, that can lead to permanent lung damage.
When to see a doctor
Contact your child's health care provider if your child tires easily. Also contact your provider if your baby is not growing or gaining weight.
Call 911 or go to the emergency room if your child has blue or grey skin or lips, faints, is hard to wake up, or has trouble breathing.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Either kind of doctor can help you keep track of follow-up visits and coordinate care with the heart specialists.
Common questions
Does double outlet right ventricle need surgery?
Yes. Treatment requires surgery to close the hole in the heart and direct blood from the left ventricle into the aorta. The type and number of operations depend on the child.
Is there a hole in the heart with double outlet right ventricle?
Yes. A ventricular septal defect, which is a hole between the two ventricles, always occurs with DORV. It lets oxygen-rich blood mix with oxygen-poor blood.
Can double outlet right ventricle be found before birth?
Yes, it can. A study found that DORV can be diagnosed prenatally with impressive accuracy.
Does double outlet right ventricle come with other heart problems?
Often, yes. People with DORV often have other heart defects, such as coarctation of the aorta, mitral valve problems, and pulmonary valve stenosis.
Sources
- MedlinePlus - Double outlet right ventricle
- National Library of Medicine (PubMed Central) - Double outlet right ventricle
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
