Double outlet right ventricle (DORV) is a heart disease that is present from birth (congenital). The aorta connects to the right ventricle (the chamber of the heart that pumps blood to the lungs), instead of to the left ventricle (the chamber that normally pumps blood to the body).
Both the pulmonary artery (which carries oxygen-poor blood to the lungs) and aorta (which carries oxygen-rich blood from the heart to the body) come from the same pumping chamber. No arteries are connected to the left ventricle (the chamber that normally pumps blood to the body).
In a normal heart structure, the aorta connects to the left ventricle, the chamber that pumps blood into the body. The pulmonary artery normally is connected to the right ventricle. In DORV, both arteries flow out of the right ventricle. This is a problem because the right ventricle carries oxygen-poor blood. This blood is then circulated throughout the body.
Oxygen-rich blood from the lungs flows from the left side of the heart, through the VSD opening and into the right chamber. This helps the infant with DORV, because it allows oxygen-rich blood to mix with blood lacking in oxygen. Even with this mixture, the body may not get enough oxygen. This makes the heart work harder to meet the body’s needs. There are several types of DORV.
The difference between these types is the location of the VSD compared to the location of the pulmonary artery and aorta. The symptoms and severity of the problem will depend on the type of DORV the baby has. The presence of pulmonary valve stenosis also affects the condition.
People with DORV often have other heart abnormalities, such as:
Symptoms of DORV may include:
Signs of DORV may include:
Tests to diagnose DORV include:
Treatment requires surgery to close the hole in the heart and direct blood from the left ventricle into the aorta. Surgery may also be needed to move the pulmonary artery or aorta.
Factors that determine the type and number of operations the baby needs include:
How well the baby does depends on:
Complications from DORV may include:
Children with this congenital heart disease may need to take antibiotics before dental treatment. This prevents infections around the heart. Antibiotics may also be needed after surgery.
Call your health care provider if your child seems to tire easily, has trouble breathing, or has bluish skin or lips. You should also consult your health care provider if your baby is not growing or gaining weight.
DORV; Taussig-Bing anomaly; DORV with doubly-committed VSD; DORV with noncommitted VSD; DORV with subaortic VSD
Baldwin HS, Dees Ellen. Embryology and physiology of the cardiovascular system. In: Gleason CA, Devaskar S, eds. Avery's Diseases of the Newborn. 9th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 50.
Updated by: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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