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Double outlet right ventricle

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).

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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).

Alternative Names

DORV; Taussig-Bing anomaly; DORV with doubly-committed VSD; DORV with noncommitted VSD; DORV with subaortic VSD

Causes

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.

Another defect called a ventricular septal defect (VSD) always occurs with DORV. Other conditions that may be part of the defect are pulmonary valve stenosis and transposition of the great arteries .

Double outlet right ventricle

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

Symptoms of DORV may include:

  • Baby tires easily, especially when feeding
  • Bluish color of the skin and lips
  • 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 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:

  • The type of DORV
  • The severity of the defect
  • The presence of other problems in the heart
  • The child's overall condition

Outlook (Prognosis)

How well the baby does depends on:

  • 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

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.

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.

Updated: 5/14/2014

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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