Double outlet right ventricle (DORV) is a congenital heart disease in which the aorta rises from the right ventricle (the chamber of the heart that pumps blood to the lungs), instead of from the left ventricle (the normal pumping chamber 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 arise from the left ventricle (the normal pumping chamber to the body).
DORV; Taussig-Bing anomaly; DORV with doubly-committed VSD; DORV with noncommitted VSD; DORV with subaortic VSD
Causes, incidence, and risk factors
Normally, the aorta arises from the left ventricle (the chamber of the heart that usually pumps blood to the body). The pulmonary artery normally arises from the right ventricle.
The presence of a VSD helps the infant with DORV, because oxygen-rich blood from the lungs flows from the left side of the heart, through the VSD opening and into the right chamber, mixing with the oxygen-poor blood. However, the body may still not get enough oxygen even with this mixture, and the heart has to work harder to try to bring more oxygen-rich blood to the body.
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 type of DORV, and the presence or absence of pulmonary valve stenosis, affect the severity of signs and symptoms the baby may have.
Patients with DORV often have other heart abnormalities such as:
How well the baby does depends on several factors:
The size of the VSD
The size of the pumping chambers
The location of the aorta and pulmonary artery
The presence of other complications (such as coarctation of the aorta and mitral valve problems)
The baby's overall health at the time of diagnosis
Whether lung damage has occurred from too much blood flowing to the lungs for a long period of time
Complications from DORV may include:
Congestive heart failure (CHF)
High blood pressure in the lungs
Irreversible damage to the lungs due to untreated high blood pressure in the lungs
All children with this congenital heart disease should take antibiotics before dental treatment. This prevents infections around the heart. Antibiotics may also be needed after surgery.
Calling your health care provider
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.
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.
Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.