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 pumpng 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).

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. Nearly always, DORV also includes the presence of a ventricular septal defect (VSD). Pulmonary valve stenosis 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.

In addition, because the pulmonary artery receives blood from both ventricles instead of just the right one, blood pressure in the lungs increases. In other cases, because the pulmonary valve and arteries are blocked or narrow, the lungs may not get enough blood.

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
• Right aortic arch

Some may have abnormal coronary arteries (blood vessels that supply the heart muscle).

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

Signs of DORV may include:

• Enlarged heart
• Heart murmur
• High blood pressure in the lungs
• Rapid breathing
• Rapid heartbeat

Tests to diagnose DORV include:

• Chest x-rays
• Passing a thin, flexible tube into the heart to examine the arteries (cardiac catheterization)
• Ultrasound exam of the heart (echocardiogram)
• Using magnets to create images of the heart (MRI)

Treatment requires surgery to close the holes in the heart. Surgery may also be needed to fix other existing problems in the heart.

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: Congenital heart defect corrective surgery

How well the baby does depends on several factors:

• The size of the VSD
• Its location
• The size of the pumping chambers
• The presence of additional complications (such as coarctation of the aorta and mitral valve problems)
• The baby's overall health at the time of diagnosis
• Whether irreversible lung damage has occurred

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 congenital heart disease should take antibiotics before dental treatment. This prevents infections around the heart, a common problem with heart disease.

Behrman RE, Kliegman RM, Jenson HB, eds. Behrman: Nelson Textbook of Pediatrics, 17th ed. Philadelphia, PA: Saunders Elsevier; 2004:1524.

Park MK. Park: Pediatric Cardiology for Practitioners, 5th ed. Philadelphia, PA: Mosby Elsevier; 2008:287-292.

Townsend Jr. CM, Beauchamp RD, Evers BM, et al, eds. Townsend: Sabiston Textbook of Surgery, 18th ed. Philadelphia, PA: Saunders Elsevier; 2008:1772.