Truncus Arteriosus

Overview

During a baby’s development, a blood vessel exiting the heart separates into two major arteries: the pulmonary artery and the aorta. If the vessel fails to divide, the baby is born with a rare congenital heart defect called truncus arteriosus. Surgical treatment, which usually occurs during infancy, separates the pulmonary artery from the aorta. A tube called a conduit is then placed to correct blood flow. In most patients, a ventricular septal defect — an abnormal hole in the heart — is also present and patched during the surgical restructuring of the heart.

Symptoms

Although it’s rare for a newborn to survive without prompt surgical repair, untreated truncus arteriosus can cause the following symptoms in adults:

  • Clubbed fingers
  • Cyanosis — blue-tinted skin discoloration
  • Fatigue
  • Irregular heart rhythm
  • Shortness of breath
  • Swelling

Adults treated for truncus arteriosus may experience the following symptoms due to related complications:

  • Decreased endurance
  • Dizziness
  • Exercise intolerance
  • Irregular heart rhythm
  • Pulmonary hypertension
  • Shortness of breath

Prevention

It’s unknown how to prevent this congenital condition, but early treatment of any related issues can help patients avoid future complications.

Risk Factors

Adults are at risk of additional complications only because of the truncus arteriosus that was present at birth.

Diagnosis

Diagnosis is typically made during prenatal testing or through clinical evaluation shortly after birth. A cardiologist specializing in congenital heart defects, like those available at the Kentucky Adult Congenital Heart Program, may diagnose additional related complications in adults during ongoing monitoring.

Treatment

  • Medications. Adults born with truncus arteriosus defect may need medication to aid their heart’s pumping ability and address any heart rhythm issues. Other medications needed to manage blood pressure may be prescribed as well.
  • Surgical treatment. Although surgical repair is typically performed during infancy, additional monitoring and surgical treatment are often required to accommodate the body’s changes and any related complications. Over time, patients may need to repair or replace the conduit due to a variety of factors, such as blood vessel narrowing or the patient outgrowing the conduit.

Follow-up Care

Patients need to schedule regular appointments with a cardiologist to monitor their heart health throughout life. The cardiologist may use the following to track a patient’s heart function:

  • Echocardiograms use ultrasound to visualize the size of the heart and its functional capacity. • Electrocardiograms can diagnose heart rhythm abnormalities.
  • Exercise stress tests determine how the heart reacts to stress, usually while the patient walks on a treadmill 
  • Holter monitor tests use devices worn for 24 to 48 hours to assess the heart’s function over time.

Patients should also consult with an adult congenital heart disease specialist to determine if a surgical procedure is needed.

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