Double Outlet Right Ventricle

Double outlet right ventricle (DORV) means there is a heart problem. It is a heart condition that some babies are born with. There are many different types of DORV. Some babies are critically ill from the moment they are born, and some have so few symptoms it may be hard to tell that they have a problem.

The normal heart is divided into 4 parts:

  • Upper.

  • Lower.

  • Right.

  • Left.

The 2 upper chambers of the heart, called the right atrium and left atrium, receive blood as it comes back to the heart. The blood is then sent to the lower chambers. The 2lower chambers, called the right ventricle and left ventricle, pump the blood out of the heart.

The right ventricle pumps blood to the lungs, and the left ventricle pumps blood to the body. The blood leaves the ventricles by 2 different blood vessels. However, in DORV, both of these blood vessels come from the same pumping chamber, the right ventricle.

How DORV affects your baby depends on the other problems in the heart. These other problems may include:

  • Holes in the walls that divide the right and left sides of the heart.

  • Blood vessels that are too small or may be missing valves.

  • Narrowing in the aorta (coarctation).

  • Having only 1 ventricle instead of 2.

Every baby is unique. You should talk to your baby's heart specialist (cardiologist) so that you can understand what other conditions your baby's heart has, how the heart can be fixed, and which problems will need surgery.

CAUSES

DORV occurs at about week 7 in the womb. There are some genetic conditions that are associated with DORV, but many times no cause can be found. Your baby should be tested for genetic causes, and you should ask the cardiologist if your baby has a geneticsyndrome.

SYMPTOMS

Because the spectrum of DORV is so broad, it is difficult to say how your baby will act. In general, DORV falls into 2 broad categories:

  • Cyanotic, with the symptoms of:

  • Blue color in the lips or fingertips because of less oxygen in the blood.

  • Rounded fingertips and toes.

  • Worsening blueness when crying.

  • Acyanotic, with the symptoms of:

  • Pink color because of too much blood with oxygen.

  • Fast or hard breathing.

  • Fatigue or sweating when eating.

  • Sleeping more because of easy fatigue.

Both cyanotic and acyanotic babies may have difficulty gaining weight. These babies may need special formula or feeding tubes to help them eat enough. Discuss your baby's individual condition with your cardiologist so that you can know whether your baby is doing well with his or her specific problem.

DIAGNOSIS

To determine whether a baby has DORV, yourcaregiver will do most, if not all, of the following tests:

  • Exam of your baby.The caregiver will check the oxygen level in the blood, blood pressure, listen to the heart and lungs, and check for other possible findings, including evidence of a syndrome.

  • Chest X-ray. This will check the baby's heart size and shape, and it will show how much blood is flowing to the lungs.

  • Electrocardiography (EKG). This test records the electrical activity of the heart and looks for disturbances in heart rhythm (arrhythmia).

  • Echocardiography. This is an ultrasound of the heart that shows how the blood flows in the heart. There is no radiation in an echocardiogram.

  • Cardiac catheterization. A cardiologist uses a flexible tube (catheter) that goes inside the heart to check the oxygen level and blood pressure in all the parts of the heart. The baby will be asleep during this procedure. A cardiac catheterization does have radiation. It is a continuous X-ray.

TREATMENT

All babies with DORV require heart surgery. Because there are many different types of DORV, there are many different surgeries that can be done.

Until the time of surgery, your baby may be on medicines to help the heart. These medicines include:

  • Diuretics. These help the body lose excess fluid that may be building up in the lungs. Your baby will urinate more if he or she is taking a diuretic.

  • Blood pressure medicine. This will lower the baby's blood pressure to help blood flow in a more useful way in the heart.

  • Digoxin. This helps the heart pump and can also treat some heart rhythm problems.

  • Prostaglandin. If your baby's lungs are not receiving enough blood, this medicine will be used to open up an extra blood vessel to help. This is an intravenous (IV) medicine that needs to be given continuously and always in an intensive care unit.

The surgery your baby will have depends on the anatomy of the heart. The surgeon and cardiologist must explain the surgery to you in detail before the baby can go to the operating room. Some babies simply require a patch to close a hole between the 2 ventricles. Some babies require multiple surgeries throughout infancy and childhood.

PROGNOSIS

Because of the variability in the anatomy, the prognosis is variable as well. Most often, your child will need to take medicines throughout his or her life. If your baby has a syndrome, the prognosis may be different than it would be for a baby without a syndrome. As advances in medicine and surgery emerge, new treatment options become available. Be sure to keep open communication with your baby's cardiologist. The more you understand about your baby's heart, the better.