Truncus arteriosus is a congenital (present at birth) defect that occurs due to abnormal development of the fetal heart during the first eight weeks of pregnancy. The heart begins as a hollow tube, and the chambers, valves, and great arteries develop throughout the first eight weeks of pregnancy. The aorta and pulmonary artery start as a single blood vessel, which eventually divides and becomes two separate arteries. Truncus arteriosus occurs when the single great vessel fails to separate completely, leaving a connection between the aorta and pulmonary artery.
Truncus arteriosus is a complex defect where there is a single (normally there are two separate arteries) vessel arising from the heart that forms the aorta and pulmonary artery. Another congenital heart defect that occurs with truncus arteriosus is a ventricular septal defect (ventricular septum, or dividing wall between the two lower chambers of the heart known as the right and left ventricles).
Normally, there are two separate arteries (the aorta and the pulmonary artery. Oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped through the aorta out to the body.
In truncus arteriosus, oxygen-poor (blue) and oxygen-rich (red) blood mix back and forth through the ventricular septal defect. This mixed blood then flows through the common truncal vessel. Some of it will flow through the branch that becomes the pulmonary artery and on to the lungs, and some of the mixed blood will go into the aortic branch and continue to the body. The mixed blood that goes to the body does not have as much oxygen as normal, and will cause varying degrees of cyanosis (blue color of the skin, lips, and nail beds).
Truncus arteriosus occurs is uncommon and affects 1 percent to 4 percent of all cases of congenital heart disease.
Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Other times this heart defect occurs sporadically (by chance), with no clear reason for its development.
The blood that passes through the common truncal vessel has a lower oxygen content than normal. Oxygen-poor (blue) blood from the right ventricle and oxygen-rich (red) blood from the left ventricle mix together before entering the common vessel. Some of this mixed blood will go into the aorta and on to the body, producing cyanosis (blue color of the skin, lips, and nail beds).
The pulmonary artery section of the common vessel gets more blood flow than the aorta does, because the pressure is lower in the lungs than the body and it is easier for blood to travel in that direction. If not repaired, the blood vessels in the lungs become damaged by the extra blood flow. As the pressure in the blood vessels in the lungs becomes higher, less blood goes to the lungs and more goes to the body. Cyanosis becomes worse as blood with lower amounts of oxygen travels to the body.
The following are the most common symptoms of truncus arteriosus. However, each child may experience symptoms differently. Symptoms may include:
The symptoms of truncus arteriosus may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.
Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the heart defects. Symptoms your child exhibits will also help with the diagnosis.
A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. Diagnostic testing for congenital heart disease varies by the child's age, clinical condition, and institutional preferences. Some tests that may be recommended include the following:
Specific treatment for truncus arteriosus will be determined by your child's physician based on:
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:
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:
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.
Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable at home. Your child's physician 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 physicians and the hospital staff.
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 replace the valve.
Your child's cardiologist will recommend that antibiotics be given to prevent bacterial endocarditis because of the homograft or conduit.
Sometimes, even when the defect is repaired early, pulmonary hypertension becomes progressively worse. Children or adults may experience shortness of breath, decreased exercise endurance and sometimes headaches and dizziness.
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 physician regarding the specific outlook for your child.
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