Truncus Arteriosus

At Children’s Heart Center, we appreciate how worried you may be when your child receives a diagnosis of truncus arteriosus during your pregnancy or early in infancy.  It is also possible that if the condition is not obvious in utero, that you, the nurses, or your doctor may become aware of symptoms later on.

In all instances, an accurate diagnosis is the first step toward treating your child’s truncus arteriosus. That’s what we’re here for.

About Truncus Arteriosus

Truncus arteriosus is a congenital (present at birth) heart defect that affects the flow of blood from your baby’s heart to the rest of the body. This condition occurs when an early-stage large blood vessel (common truncus) does not divide normally into two separate arteries as the fetus develops.

Instead of having a pulmonary artery to carry oxygen-poor (blue) blood to the lungs to be oxygenated and an aorta to carry the oxygen-rich (red) blood to the body, your baby has one common artery that carries all the blood away from the heart together. This forces the oxygen-poor and oxygen-rich blood to mix and send too much blood to the lungs. With truncus arteriosus, there is also a large hole, a ventricular septal defect (VSD), between the two pumping lower chambers of the heart (the ventricles).

Truncus arteriosus affects your baby in two ways. Your baby will have lower oxygen levels than normal. And your baby may develop symptoms of congestive heart failure since there is an increase in blood flow to the lungs.

There are several variations of truncus arteriosus, such as:

  • Pulmonary arteries sometimes may arise from different locations on the aorta.
  • Often, the truncal valve (the single valve between the heart and the common truncus) is abnormally narrow or leaky.
  • Rarely, the aortic arch, a part of the aorta, the major blood vessel that supplies the body with blood, is interrupted and affects the blood flow to the lower half of the body. 

Symptoms

Symptoms of truncus arteriosus may include your baby having:

  • Blue or dusky lips, skin and nails
  • Difficulty feeding or poor weight gain
  • Heart murmur that can be heard with a stethoscope
  • Rapid breathing or shortness of breath 

Diagnosis

If truncus arteriosus is diagnosed with a fetal ultrasound or an echocardiogram before your baby is born, our Fetal Heart Program team will guide you through the diagnostic process and support you before, during, and after delivery. Our sole purpose is to help you prepare for delivery and to provide the right care for your infant after birth.

We may make the diagnosis of truncus arteriosus either during fetal life, following delivery of your newborn, or early during infancy.  We will order special diagnostic tests, which may include some or all of the following:

  • Pulse oximetry—a noninvasive way to monitor the oxygen content of the blood
  • Echocardiogram—a safe, noninvasive procedure that uses high frequency ultrasound to show the structure of your child’s heart, how blood flows through the heart, and overall how your child’s heart and circulatory system are working
  • Electrocardiogram (ECG or EKG)—measures electrical activity which shows the heart’s chambers and how your child’s heart is beating
  • Chest X-ray—an image that can show the position and size of your child’s heart

In addition, if we diagnose truncus arteriosus for your baby during pregnancy, as your fetal cardiologist along with your obstetrician, we will offer you the option of genetic testing.

There are genetic abnormalities associated with this diagnosis. One of the more common conditions is DiGeorge syndrome, also known as 22q11 deletion, which is associated with certain heart defects, as well as learning disorders, poor immune function, cleft palate, and low calcium levels.

Treatments We Offer

We discuss every aspect of your child’s care with you. And since every child is unique, the details of our approach will be specific for your child.

Often, we need to perform heart surgery to treat truncus arteriosus. Typically, we perform the procedure within the first weeks of life, usually before your baby leaves the hospital.

During the procedure, our pediatric cardiac surgeon detaches the pulmonary arteries from the common arterial trunk and connects them to the right ventricle (lower pumping chamber of the heart) using a conduit, or tube. This closes the VSD (the hole between the bottom chambers) to direct blood from the left lower pumping chamber (ventricle) to the aorta.

If your child has associated issues, such as significant narrowing or leaking of the truncal valve, which will become the aortic valve, or if there is aortic arch interruption, we address those issues during the same operation.

It is important for you to know that your child who has had surgical repair of truncus arteriosus will require lifelong care by a cardiologist. Over time, we will monitor the function of the truncal valve and heart.  Also, since the conduit or tube used to connect the right ventricle to the pulmonary arteries does not grow as your child does, we will eventually need to replace the tube with a larger size. 

Through it all, we are here for you and your child for life. Our goal is to help your child be a child. 

Our Alliance with Children’s Hospital of Philadelphia

Children’s Heart Center is an alliance between Mount Sinai and Children’s Hospital of Philadelphia (CHOP). This innovative alliance brings together the expertise of two world-leading institutions for the best possible outcomes. Together, we offer the most effective treatment and compassionate cardiac care for your child here in New York City.

Why Choose Children’s Heart Center?

Children’s Heart Center team of compassionate and experienced pediatric cardiologists provide the type of personal, sensitive, and expert care your baby needs.

We specialize in detecting, evaluating, and managing congenital heart defects like truncus arteriosus. We offer the most accurate diagnostics and outstanding treatment outcomes. We empathize with you and communicate one-on-one with you about your baby’s condition. Each baby is special and unique to us.

Our staff makes sure that you understand what is going on during diagnostic tests and any required surgery. We are here to treat your child and support you in focusing on caring for your child and the rest of your family.

Rest assured. At Children’s Heart Center, we provide your child with the finest care.