Treatment for truncus arteriosus
Specific treatment for truncus arteriosus will be determined by your child's doctor based on:
Your child's age, overall health, and medical history
Extent of the condition
Your child's tolerance for specific medications, procedures, or therapies
Expectations for the course of the condition
Your opinion or preference
Truncus arteriosus must be treated by surgical repair of the defects. However, medical support may be necessary until the best time for the operation to take place. Treatment may include:
Medical management. Many children will eventually need to take medications to help the heart and lungs work better. Medication that may be prescribed includes the following:
Digoxin. A medication that helps strengthen the heart muscle, enabling it to pump more efficiently.
Diuretics. The body's water balance can be affected when the heart is not working as well as it could. These medications help the kidneys remove excess fluid from the body.
ACE (angiotensin-converting enzyme) inhibitors. Dilates the blood vessels, making it easier for the heart to pump blood forward into the body (instead of into the lungs).
Adequate nutrition. Infants may become tired when feeding, and may not be able to eat enough calories to gain weight. Options that can be used to ensure your baby will have adequate nutrition include:
High-calorie formula or breast milk. Special nutritional supplements may be added to formula or pumped breast milk that increase the number of calories in each ounce, thereby allowing your baby to drink less and still consume enough calories to grow.
Supplemental tube feedings. Feedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, can either supplement or take the place of bottle-feedings. Infants who can drink part of their bottle, but not all, may be fed the remainder through the feeding tube. Infants who are too tired to bottle-feed may receive their formula or breast milk through the feeding tube alone.
Surgical repair. Surgery is usually performed within the first few weeks after birth, before the blood vessels in the lungs are overwhelmed by extra blood flow and become diseased.
The operation is performed under general anesthesia, and involves the following:
The pulmonary arteries are detached from the common artery (truncus arteriosus) and connected to the right ventricle using a homograft (a section of pulmonary artery with its valves intact from a tissue donor). Occasionally, a conduit (a small tube containing a valve) is used instead of a homograft (human tissue valve).
The ventricular septal defect is closed with a patch.
Postoperative care for your child
Children will spend time in the intensive care unit (ICU) after a truncus repair.
While your child is in the ICU, special equipment will be used to help him or her recover, and may include the following:
Ventilator. A machine that helps your child breathe while he or she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he or she is too sleepy to breathe effectively on his or her own. After a truncus repair, children will benefit from remaining on the ventilator overnight or even longer so they can rest.
Intravenous (IV) catheters. Small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medicines that help your child recover from the operation.
Arterial line. A specialized IV placed in the wrist or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the ICU.
Nasogastric (NG) tube. A small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.
Urinary catheter. A small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and, therefore, the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys remove excess fluid from the body.
Chest tube. A drainage tube may be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.
Heart monitor. A machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values.
Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.
Your child will be kept as comfortable as possible with several different medications; some of which relieve pain, and some of which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.
After discharge from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while at home, and these will be explained to you. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.
Caring for your child at home following truncus arteriosus repair
Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable at home. Your child's doctor will discuss pain control before your child is discharged from the hospital.
Often, infants who fed poorly prior to surgery have more energy after the recuperation period, and begin to eat better and gain weight faster. However, high-calorie formulas may be needed for several weeks or months after surgery to help your child catch up growth-wise. Tube feedings may also be helpful until your child is able to feed better.
After surgery, older children usually have a fair tolerance for activity. Your child may become tired easily, and sleep more right after surgery, but, within a few weeks, your child should be fully recovered.
You may receive additional instructions from your child's doctors and the hospital staff.
Long-term outlook after truncus arteriosus surgical repair
Many children who have had truncus arteriosus surgical repair can live healthy lives. Activity levels, appetite, and growth will eventually return to normal in most children.
Future intervention may be necessary if the pulmonary artery branches were small and do not grow well after surgery. The homograft or conduit connecting the right ventricle to the pulmonary artery may also need to be replaced in the future as your child grows. Surgery in young adulthood is often needed to replace the conduit and/or the valve.
Your child's cardiologist will recommend that antibiotics be given to prevent bacterial endocarditis because of the homograft or conduit.
Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individual’s lifespan.
Consult your child's doctor regarding the specific outlook for your child.