Truncus Arteriosus

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Children with truncus arteriosus are born with one large artery carrying blood to the lungs and body instead of two separate arteries.

Almost all children with truncus arteriosus (pronounced TRUNG-kus ar-teer-ee-OH-sus) also have a ventricular septal defect (VSD) — a hole in the wall between the right ventricle and left ventricles. This hole lets oxygen-rich blood mix with oxygen-poor blood and go through the single artery to the body and the lungs.

As a result of truncus arteriosus, the lungs can get too much blood. The high blood volume can damage the lungs’ blood vessels and cause the heart to pump extra hard.

The valve that sits between the heart and the large truncus artery often is abnormal as well. This valve may be leaky or tight, or even both at the same time.

As the leading providers of pediatric heart care in Louisville and Southern Indiana, our specialists are experienced in successfully repairing truncus arteriosus.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.

Most babies recover well after surgery to repair truncus arteriosus. However, some will need more operations as they grow.

Truncus Arteriosus Symptoms and Causes

As the baby develops during pregnancy, the aorta and the pulmonary artery begin as a single vessel before splitting. In some children, the split doesn’t happen because of a genetic defect — 22q11.2 deletion syndrome (also called DiGeorge syndrome). In other children, it’s not known why it happens.

Symptoms of truncus arteriosus may include a blue or purple tint to the skin, rapid or labored breathing, problems with feeding, poor weight gain, sleepiness and increased sweating, especially while eating.

Health care providers listening to the baby’s heart usually will hear a heart murmur or whooshing sound.

A child born with truncus arteriosus might not look sick right away. But if the condition isn’t treated early, truncus arteriosus can quickly lead to heart failure and other life-threatening complications.

Truncus Arteriosus Diagnosis

Doctors often can diagnose truncus arteriosus before birth. A fetal echocardiogram is a test that uses sound waves to create a video of the baby’s heart before the baby is born.

If the problem isn’t found before birth, most babies will show signs of this heart defect within the first days or weeks of life. Pulse oximetry, a simple test that measures the amount of oxygen in the bloodstream, may give the first clue that there is a heart defect. A heart ultrasound (or echocardiogram) done after birth will clearly show truncus arteriosus.

Children with truncus arteriosus are born with one large artery carrying blood to the lungs and body instead of two separate arteries.

Truncus Arteriosus Treatment

Babies with truncus arteriosus need open heart surgery to prevent complications, and most babies will not survive without surgical repair. Most babies have this surgery in the first month of life.

During truncus arteriosis repair, the aorta and pulmonary artery are separated, creating a pathway for blood to travel from the right ventricle out to the lungs. The VSD and any other heart defects are repaired at the same time.

Before surgery, medicines can help keep the baby stable. In some cases, more than one operation is needed.

In some kids, cardiac catheterization can help fix areas narrowed by scar tissue. All children with truncus arteriosus need regular visits with their doctor to help prevent future complications.

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