Double Outlet Right Ventricle Surgery

A child with double outlet right ventricle (DORV) always needs surgery to repair the heart.

In a normal heart, there are 2 main blood vessels. The pulmonary artery brings blood to the lungs. The aorta brings blood to the body. In normal hearts, these 2 blood vessels are connected to 2 separate pumping chambers. However, in DORV, they are both connected to the same chamber.

The type of surgery can vary depending on the rest of the anatomy of the heart. Some children only need one surgery. Some need multiple surgeries throughout infancy and childhood.

LET YOUR CAREGIVER KNOW ABOUT:

  • Allergies to food or medicine.

  • Medicines taken, including vitamins, herbs, eyedrops, over-the-counter medicines, and creams.

  • Use of steroids (by mouth or creams).

  • Previous problems with anesthetics or numbing medicines, both in the child and in family members.

  • History of bleeding problems or blood clots.

  • Previous surgery.

  • Other health problems.

RISKS AND COMPLICATIONS

Heart surgery is complicated and has serious risks. These risks include bleeding, infection, damage to the nerves or muscles in the chest, damage to the electrical system in the heart, strokes, and blood clots. If your child's surgery includes a heart-lung bypass machine, there is an increased risk of bleeding in the brain and difficulties with blood pressure. All surgeries have a risk of life-threatening complications.

BEFORE THE PROCEDURE

  • A medical evaluation is needed. This may include:

  • Physical exam by a heart specialist (cardiologist) and surgeon.

  • Chest X-ray. This will check the child'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 child will be asleep during this procedure. A cardiac catheterization does have radiation. It is a continuous X-ray.

  • You will need to meet with several people prior to the surgery. These include:

  • Your child's cardiologist.

  • Your child's heart surgeon.

  • The anesthesiologist.

  • A social worker who can help arrange for special equipment and supportive care that your child may need after surgery.

  • Often, the child will require a blood transfusion during and after surgery. Family members can donate blood for the surgery if you want this. You must express this wish to the surgeon.

  • On the day of surgery:

  • The child must not eat or drink anything for at least 8 hours before surgery. Usually, it is acceptable to give medicines with a small amount of water.

  • If the child is already in the hospital, an intravenous (IV) access tube will be placed the night before surgery, and fluids may be given.

  • If you are coming from home, be sure to arrive 2 hours before the scheduled surgery so that you can fill out paperwork and address any problems that might arise.

  • The hospital stay may be as short as a few days or last longer than a week. Make plans for someone to stay overnight, if possible, every night of the hospitalization.

PROCEDURE

As every heart is unique, it is difficult to predict every surgery for every child. Be sure to speak openly with your surgeon so that you understand what surgery your child is having.

In preparation for surgery:

  • The child will have an IV placed.

  • The child may receive medicine to help him or her relax (sedative) before entering the operating room.

  • Once in the operating room, the child will receive medicine that makes him or her sleep (general anesthetic). Then, the child will be put on a machine that helps him or her breathe (ventilator). A tube will be placed into the windpipe so that breathing can be controlled during surgery.

  • More IV lines will be placed, including central lines. These are larger IVs in larger veins or arteries that give the surgeon and anesthesiologist the most accurate measurement of blood pressure in the arteries and veins.

  • A small tube will be placed that goes from the nose down to the stomach (nasogastric tube).

  • The child will also have a tube that lets urine drain from the body (Foley catheter) during surgery. This tube may stay in after surgery.

  • The surgeon may also use transesophageal echocardiography. This is an ultrasound test similar to the echocardiography your child has already had, except that the probe is in the esophagus instead of on the chest. This gives the surgeon more accurate pictures of the heart before surgery.

During the surgery:

  • A cut (incision) is made. Most commonly, the incision is made through the breastbone, but depending on the surgery the incision may be under the arm.

  • The child may be connected to a heart-lung bypass machine. This supplies the body with oxygen while the heart is having surgery.

  • When the repairs are done, the child will be taken off the heart-lung bypass machine. The heart may require medicine to help it beat well, and this will be given by the central lines that were placed before surgery.

  • The surgeon will put small, clear chest tubes in to drain excess fluid and air from the chest.

  • The child may have temporary electrical wires placed to ensure that the heart's electricity is normal.

  • The incision will be closed and a bandage will be placed over it.

AFTER THE PROCEDURE

The child will be brought to the intensive care unit (ICU) to recover after surgery. Depending on the hospital, this may be a:

  • Specialized cardiac intensive care unit.

  • Neonatal intensive care unit (NICU).

  • Pediatric intensive care unit (PICU).

Many of the tubes and wires placed during surgery will be left in place during the first few hours or days of recovery. The child may still be on a ventilator and may need IV blood pressure medicine. The child will be attached to several monitors to check breathing, heart rate, and blood pressure.

While in the ICU, the child will have multiple X-rays to check the heart and lungs, as well as frequent blood tests. As the child recovers, he or she will require less and less monitoring. Tubes will be removed, and the child will be taken off of the ventilator to breathe on his or her own.

The amount of time spent in the hospital after surgery depends on which surgery was performed. Some patients can be discharged within 2 to 3 days. Others require a longer hospital stay that could last weeks. You should discuss the expectations with the surgeon before the surgery.