What is double outlet right ventricle?

Double outlet right ventricle (DORV) is a rare congenital heart defect (the baby is born with it) involving the "great arteries" (the aorta and the pulmonary artery). In a normal heart, the aorta exits from the left ventricle and pumps blood to the body, while the pulmonary artery exits from the right ventricle and pumps blood to the lungs (where it picks up oxygen). In DORV, the aorta and the pulmonary artery both come out of the right ventricle.

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The causes of DORV are not completely understood. DORV appears in many forms, with most including a ventricular septal defect . A ventricular septal defect is a hole in the wall of the septum, the muscular wall that separates the right ventricle from the left ventricle. Cases of DORV can vary due to the size of the ventricular septal defect, where the ventricular septal defect is located, and the existence of pulmonary valve stenosis (a blockage in the flow of blood from the right ventricle to the pulmonary artery).

Having the hole in the septum is actually helpful in DORV because blood that has oxygen is able to pass through the hole from the left ventricle into the right for circulation in the body. If there were no hole, the blood could not be distributed. However, because the right ventricle normally collects oxygen-poor blood to pump to the lungs, the two types of blood mix and the baby does not get the proper levels of oxygenated blood.

What are some common types of DORV defects?

One of the ways that DORV is diagnosed is by the location of the ventricular septal defect:

  • When the ventricular septal defect is just under the aorta, the condition is called DORV with subaortic ventricular septal defect.
  • When the ventricular septal defect is just under the pulmonary artery, the condition is called DORV with subpulmonary ventricular septal defect, or Taussig-Bing anomaly.
  • When the ventricular septal defect is under both of the great arteries, the condition is called DORV with doubly committed ventricular septal defect.
  • When the ventricular septal defect is not near the aorta or the pulmonary artery, the condition is called DORV with non-committed ventricular septal defect or DORV with remote ventricular septal defect.

Another factor in determining the type of DORV is whether or not the condition includes pulmonary valve stenosis and how extensive the stenosis is. In addition, an infant can have DORV along with other types of congenital heart defects.

What are the symptoms of DORV?

DORV symptoms are similar to those of other types of congenital heart defects and illnesses. Symptoms may include:

  • A heart murmur (an extra, unusual heart sound)
  • Breathing problems, including difficulty breathing, shortness of breath, or rapid breathing
  • Difficulty eating and/or gaining weight
  • Blueness of the lips, nails, and/or skin (cyanosis)
  • Sweating
  • Fatigue

How is dorv diagnosed?

Several tests may be used to diagnose DORV. The first is a physical examination, during which the doctor uses a stethoscope to listen to the heartbeat and hears a heart murmur. Further tests to diagnose heart defects might include:

  • Chest X-ray
  • Electrocardiogram to test heart rate patterns
  • Echocardiogram to take pictures of the heart using sound waves
  • Cardiac catheterization (a thin tube is inserted into a vein and advanced to the heart) to obtain details on heart function

DORV might also be discovered while the mother is pregnant.

How is DORV treated?

Most cases of DORV require surgery, although this will be determined by the doctor and the parents. Factors to consider include age, overall health, and expected recovery. The goal of surgery is to connect the aorta to the left ventricle and the pulmonary artery to the right ventricle.

One type of surgery uses the existing ventricular septal defect to create a tunnel (called a baffle) to connect the left ventricle to the aorta. The connection occurs through the ventricular septal defect. This type of surgery is called intraventricular repair.

Another type of surgery is called an arterial switch. In this surgery, a tunnel is also made from the ventricular septal defect to the pulmonary artery. This connects the pulmonary artery to the left ventricle. A second stage involves switching the great arteries from this backward position. In the second stage, the aorta is disconnected and then reconnected to the left ventricle and the pulmonary artery is moved to connect to the right ventricle. Coronary arteries are also moved to connect with the aorta.

Can DORV be prevented?

In most cases, there is no way to prevent congenital heart defects. However, there are some things that can be done to make pregnancies healthier:

  • A pregnant woman should not drink alcohol or take drugs that have not been prescribed to her.
  • A woman with certain chronic conditions (like diabetes, a seizure disorder, or phenylketonuria) can consult her doctors before conception for advice on medications or special diets.
  • A woman who is pregnant should get 400 micrograms of folic acid (folate) per day to prevent birth defects.

What is the prognosis (outlook) for children with DORV?

The outlook for children with DORV who have surgery is generally good. However, factors that affect recovery include the severity of the defect, the amount of damage already done to the lungs, and the overall health of the baby.

As with other congenital heart defects, people with DORV are at greater risk for developing infections of the heart and valves. They may have to take antibiotics when having certain dental or surgical procedures.

This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 1/13/2011…#14733

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