This rare birth condition occurs when two of the heart’s most important arteries and their respective valves don’t form separately.

Normally, the aorta carries blood from the heart to the body, and the pulmonary artery carries blood to the lungs. But in truncus arteriosus, these arteries don’t form separately. A baby is born with just one large, common artery (“truncus artery”) to carry blood to both the body and lungs.

There are four different types of truncus arteriosus, depending on exactly how the arteries form. Most babies with truncus arteriosus are also born with a ventricular septal defect (VSD), a hole in the wall between the lower left and lower right chambers of the heart.

As a result of all of this, blood mixes in the heart in a way that it shouldn’t, and too much blood can flow to the lungs and not enough to the body. This damages the lungs and strains the heart.

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What Are the Signs and Symptoms of Truncus Arteriosus?

Truncus arteriosus is often detected before birth, or in the first few days of a baby’s life. The most common signs are similar to many other heart problems.

  • Heart murmur
  • Bluish or grayish lips, skin and nails
  • Trouble breathing
  • Trouble feeding
  • Sweating, especially during feeding
  • Poor weight gain
  • Low energy and activity

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What Causes Truncus Arteriosus?

Truncus arteriosus occurs before birth, when a baby’s heart doesn’t develop properly during the first eight weeks of pregnancy.

In some babies, it may be linked to one of these risks.

  • Genetic condition like DiGeorge syndrome
  • Family history of congenital heart problems
  • Mother had an infection like rubella (German measles) during pregnancy
  • Mother used certain harmful medications during pregnancy

But truncus arteriosus also occurs in babies without any of the above risk factors, without any known cause.
 

How is Truncus Arteriosus Treated?

Most babies with truncus arteriosus have heart surgery within their first few weeks of life. Connecticut Children’s congenital cardiac surgeons specialize in this procedure, and are known for outstanding surgical outcomes.

  • Repair of truncus arteriosus: Open-heart surgery to separate and reconstruct the large artery into the correct arteries; place a right ventricle to pulmonary artery (RV-PA) conduit; and repair the VSD, valve problems, and any other heart problems

Patients born with truncus arteriosus need congenital heart care that continues into adulthood. Along with routine follow-up care and tests, they often need another surgery or catheter procedure to swap in a larger RV-PA conduit.

Connecticut Children’s Heart Center provides this lifelong care and expertise with our nationally accredited Adult Congenital Heart Disease program.