What is truncus arteriosus?
Truncus arteriosus is a rare type of congenital birth defect in which the two major arteries of the heart (the aorta and the pulmonary artery) fail to separate during development. Instead, there is only one large vessel coming from the heart.
The valve arising from the single blood vessel, called the truncal valve, is often abnormal. The valve can be thickened and narrowed which can block the blood as it leaves the heart. The truncal valve can also leak, causing blood that leaves the heart to leak back into the heart across the valve.
The large artery, known as the truncus arteriosus, has one valve that carries blood from both ventricles. Almost every child with this heart condition will also have a hole between the ventricles, known as a ventricular septal defect (VSD). This condition results in too much blood going to the lungs and can result in irreversible damage to the heart and the arteries of the lungs.
Who is at risk for truncus arteriosus?
Truncus arteriosus is a congenital heart defect, meaning it develops as a child is growing in the womb. While doctors do not know exactly what causes truncus arteriosus, there are several common risk factors that may increase the chances of a baby being born with a heart condition.
Prenatal diagnosis is important, as babies with truncus arteriosus may have additional organ or chromosomal abnormalities such as DiGeorge syndrome, also known as 22q11.2 Deletion Syndrome. This and other genetic syndromes require specialized treatment from the experts at the Colorado Fetal Care Center and Children's Hospital Colorado.
What are signs of truncus arteriosus?
Children with truncus arteriosus are usually diagnosed early in life. Sometimes children have blue skin and lips, called cyanosis, which is a sign that not enough oxygenated blood is getting through the body. You should call your doctor immediately if you notice signs of cyanosis.
Other truncus arteriosus symptoms include:
- Shortness of breath, especially during feeding
- Sleepiness and fatigue
- Poor weight gain
- Sweatiness, especially during feeding
How is truncus arteriosus diagnosed?
Your doctor may hear a murmur when listening to your child's heart during a physical exam. If your doctor suspects your child might have truncus arteriosus, he or she will order further tests.
An echocardiogram (ECHO) is the best test to diagnose truncus arteriosus. This test will give cardiologists at Children's Colorado a moving picture of your child's heart in action.
Sometimes, a chest X-ray will also be done to see if your child's lungs have fluid in them.
Truncus arteriosus treatment at Children's Colorado
If your child has been diagnosed with truncus arteriosus, he or she will need heart surgery to repair it.
Surgery is recommended within the first few weeks of life if the condition is detected immediately after birth. During the procedure, a pediatric cardiac surgeon will close the holes within the heart and separate the pulmonary and truncal arteries.
Usually, surgeons will place a tube or conduit from the right ventricle to the pulmonary artery. This tube will need to be replaced in the future, usually several years after the first operation as the child grows.
In older children, surgical therapy may not be possible, which is why it is important to diagnose this condition as soon as possible.
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Recovery and life after heart surgery
After truncus arteriosus treatment, children will spend 2 to 3 weeks in the hospital and have follow-up appointments with a cardiologist.
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