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Double outlet right ventricle
Definition
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).
Alternative Names
DORV; Taussig-Bing anomaly; DORV with doubly-committed VSD; DORV with noncommitted VSD; DORV with subaortic VSD
Causes
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.
In DORV, both arteries arise from the right ventricle. This is a problem because the right ventricle carries oxygen-poor blood, which the aorta then carries throughout the body. DORV always includes a ventricular septal defect (VSD). Pulmonary valve stenosis or transposition of the great arteries may also be part of the defect.
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:
- Coarctation of the aorta
- Mitral valve problems
- Pulmonary atresia
- Pulmonary valve stenosis
- Right aortic arch
- Transposition of the great arteries
Symptoms
Symptoms of DORV may include:
- Baby tires easily, especially when feeding
- Bluish skin color (the lips may also be blue)
- Clubbing (thickening of the nail beds) on toes and fingers (late sign)
- Failure to gain weight and grow
- Pale coloring
- Sweating
- Swollen legs or abdomen
- Trouble breathing
Exams and Tests
Signs of DORV may include:
- Enlarged heart
- Heart murmur
- Rapid breathing
- Rapid heartbeat
Tests to diagnose DORV include:
- Chest x-rays
- Passing a thin, flexible tube into the heart to measure blood pressure and inject dye for special pictures of the heart and arteries (cardiac catheterization)
- Ultrasound exam of the heart (echocardiogram)
- Using magnets to create images of the heart (MRI)
Treatment
Treatment requires surgery to close the hole in the heart and properly direct blood from the left ventricle into the aorta. Surgery may also be needed to move the pulmonary artery or aorta.
Several factors determine the type and number of operations the baby needs. They include:
- The type of DORV
- The severity of the defect
- The presence of other problems in the heart
- The child's overall condition
See also:
Outlook (Prognosis)
How well the baby does depends on several factors:
- The size of the VSD
- Its location
- 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
Possible Complications
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.
When to Contact a Medical Professional
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.References
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.
Reviewed By: 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.
