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 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).

Alternative Names

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.

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.

In addition, because the pulmonary artery receives blood from both ventricles instead of just the right one, blood pressure in the lungs increases.

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:

• Anomalous coronary arteries
• 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

Signs 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 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
• Pediatric heart surgery

Support Groups

Expectations (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 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

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.

Calling your health care provider

Prevention

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References

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.