Definition

Double-outlet right ventricle (DORV) is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood moves out to the rest of the body.

Normally, the aorta (the largest artery) comes from the heart’s left ventricle. But when DORV occurs, the aorta and the pulmonary artery are attached to the right ventricle.

Another heart defect called ventricular septal defect (VSD) usually occurs with DORV. VSD is a hole in the wall between the right and left ventricle. DORV can be categorized into several types depending on the position of the VSD.

Along with these conditions, the pulmonary valve may be narrowed. DORV is a serious condition. It requires care right away by the doctor.

Heart Chambers and Valves

heart anatomy

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Blood Flow Through the Heart

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Causes

This condition is a congenital defect. This means that the heart forms incorrectly when the baby is developing in the womb. The baby is born with the condition. It is not known exactly why the heart develops abnormally in some babies.

Risk Factors

For many heart defects, the risk factors are unclear. Some risk factors for DORV may include:

    
  • Family history of congenital heart defect
  • Certain chromosomal disorders

    • Symptoms

    Symptoms may vary depending on where the VSD is located. Symptoms may include:

        
  • Bluish skin color
  • Poor feeding/slow weight gain
  • Fatigue
  • Sweating
  • Rapid breathing
  • Shortness of breath

    • During the exam, the doctor may also detect a heart murmur .

    These symptoms may be due to other conditions. If your child has any of these, talk to the doctor right away.

    Diagnosis

    Your doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:

        
  • Chest x-ray —an imaging test that takes a picture of the heart and lungs
  • Electrocardiogram (EKG)—a test that measures the electrical activity of the heart
  • Echocardiogram —an imaging test that uses sound waves to look at the size, shape, and motion of the heart
  • MRI scan —a test that uses magnetic waves to make pictures of structures inside the chest
  • Cardiac catheterization —a test that uses a catheter (tube) and x-ray machine to assess the heart and its blood supply

    • Treatment

    Talk with the doctor about the best treatment plan for your child. Often, surgery is needed right away. Treatment options include:

    Surgery

    Surgery can be done to correct DORV. The goal of surgery is to connect the aorta to the left ventricle. Surgery can range in complexity. The doctor may insert a shunt or create a new “tunnel” to connect the left ventricle to the aorta through the VSD. Pulmonary artery banding may be used to limit blood flow to the lungs. Depending on other abnormalities, a more complex surgery may be needed to change the position of the large arteries and reconnect other vessels.

    Lifelong Monitoring

    Your child will have regular exams from a heart specialist. In some cases, your child may need antibiotics prior to some medical or dental procedures. This is to prevent an infection in the heart.

    Prevention

    There is no known way to prevent DORV. Getting appropriate prenatal care is always important.