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The Rastelli procedure is used to treat some patients with D-TGA (also called “transposition of the great arteries”). Children with this condition are born with the main blood vessels coming off the heart reversed. The pulmonary artery usually carries blue blood to the lungs to get oxygen, while the aorta usually carries red blood to the rest of the organs and tissues of the body. When these vessels are reversed, surgery is needed to allow normal blood flow.
Surgical treatment can vary depending on your child’s situation: Some patients with D-TGA might have an operation called the arterial switch or other operations. The Rastelli procedure is used to treat patients who have both transposition of the great arteries and ventricular septal defect (hole between the two main pumping chambers of the heart).
What is the Rastelli procedure?
With the Rastelli procedure, the surgeon uses a synthetic patch positioned so it closes the ventricular septal defect (VSD) in a way that directs blood from the left ventricle to the aorta. This allows red blood to go to the organs of the body. The surgeon also connects a conduit (a tube with a valve made of synthetic material or human or animal tissue) to direct blood from the right ventricle to pulmonary artery so that blood can reach the lungs for oxygenation.
Experts in the Rastelli procedure
This is a complex procedure that should only be done by a surgeon experienced with this advanced repair. The Herma Heart Institute is one of the nation’s top pediatric heart programs and consistently delivers some of the best congenital heart surgery outcomes for even the most complex types of heart disease. If your child has already been diagnosed with cyanotic heart defects at another center, we invite you to get a second opinion at the Herma Heart Institute. We’re proud of our history of innovation that has changed the practice of heart surgery worldwide. The Herma Heart Institute is also home to a leading Adult Congenital Heart Disease Program, allowing for seamless, high quality care across your child’s lifespan.
Who needs a Rastelli procedure?
This procedure is used to repair certain combinations of congenital heart defects, including:
- Transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis
- Overriding of the aorta with severe pulmonary stenosis or pulmonary atresia
- Pulmonary atresia with ventricular septal defect
- Double outlet right ventricle (DORV) with pulmonary stenosis or pulmonary atresia
- Tetralogy of Fallot (TOF)
Babies with these conditions typically have temporary repairs soon after birth with a shunt (tube) placed to increase blood flow to the lungs, and then the more complex Rastelli procedure is performed when a child is bigger and stronger, usually between ages 1-2. Your child’s doctor will advise you on the best options for your child.
What to expect during a Rastelli procedure
This surgery takes several hours while your child is under general anesthesia in the operating room. The surgeon will make an incision in your child’s chest and open the breast bone to access the heart. During the surgery, your child will be connected to a heart-lung machine that does the work of the heart and lungs. The surgeon then will patch your child’s VSD and attach the new conduit between the right ventricle and pulmonary artery. When the repairs are complete, your child will be taken off the heart-lung machine, and the surgeon will close up your child’s chest.
After surgery, your child will spend time in the cardiac intensive care unit (CICU). This is a major operation, and recovery from this surgery can take days to weeks. The first few days after surgery your child may require a ventilator to help with breathing and strong heart medications.
While your child is in the CICU, Children’s might use the following equipment:
- Ventilator - This machine helps your child breathe. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while they are under anesthesia. Many children have the ventilator tube removed right after surgery, but some will benefit from remaining on the ventilator for a few hours afterward so they can rest.
- Intravenous (IV) catheters - These small, plastic tubes inserted into blood vessels provide IV fluids and important medications that help your child recover from the operation.
- Heart lines - Small catheters are placed through the chest wall into the heart to measure blood pressure and to provide certain medicines. These are removed at the bedside when no longer needed.
- Pacer wires - Small wires are attached to the heart at the time of surgery. These are used to allow use of a pacemaker. The pacemaker can help adjust your child’s heart rate and rhythm. These are removed at the bedside when no longer needed.
- Arterial line - A specialized IV placed in the wrist, or other area of the body where a pulse can be felt, measures blood pressure continuously during surgery and while your child is in the ICU.
- Nasogastric (NG) tube - A small, flexible tube keeps the stomach drained of acid and gas bubbles that could build up during surgery.
- Urinary catheter - This small, flexible tube drains urine out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. Some swelling and puffiness is common after surgery, and your child might be given diuretics to help the kidneys remove excess fluids.
- Chest tube - A drainage tube might be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding can occur for several hours or even a few days after surgery.
- Heart monitor - This machine constantly displays a picture of your child’s heart rhythm and monitors heart rate, arterial blood pressure and other values.
Your child might need other supportive equipment while in the CICU or afterward. They will be kept as comfortable as possible with medications that relieve pain and anxiety, and the staff might also ask for your input on how best to soothe and comfort your child.
Your child will need to stay in the hospital 1-2 weeks to recover after a Rastelli procedure. After your child is discharged from the CICU, they will recuperate on another cardiac floor for a few days before going home. You will learn how to care for your child at home and will receive instructions on medications, activity limitations and follow-up appointments before your child is sent home.
Care for your child at home following a Rastelli procedure
Most children feel fairly comfortable when they go home, and your physician might recommend pain medications, such as acetaminophen or ibuprofen, to keep them comfortable. Your child might become tired more quickly than before surgery, but they usually will be allowed to play with supervision. It’s important to avoid blows to the chest that might cause injury to the incision or breastbone. Within a few weeks, your child should be fully recovered and able to participate in normal activity.
Long-term outlook and success rates of a Rastelli procedure
Rastelli procedure success rates vary depending on your child’s specific diagnosis, but this is generally considered a low-risk procedure. Many children go on to lead relatively normal lives, though they might need to avoid strenuous competitive sports. Surgically implanted conduits and valves can wear out or develop problems over time, so your child might eventually need another surgery later to repair or replace the conduit and/or valve. Your child will always need to be monitored by a cardiologist in case new problems develop, and once they reach adulthood, they should seek out a program that specializes in adult congenital heart disease like Children’s ACHD program.