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What is Truncus Arteriosus?

Truncus arteriosus is a rare congenital heart defect where only one large blood vessel leads out of the heart instead of two. Also, the wall that divides two lower chambers of the heart has a hole, which causes oxygen-rich and oxygen-poor blood to mix together. This causes problems circulating enough oxygen to the body.

After birth, a baby with truncus arteriosus develops bluish skin, trouble feeding, excessive sleepiness, breathing problems, high blood pressure in the lungs, enlargement of the heart, and a failure to thrive. If undiagnosed and untreated, truncus arteriosus can be fatal. However, with early treatment, the long-term prognosis is promising.

Truncus arteriosus is caused by chance and environmental factors. Gestational diabetes, viral illness such as rubella during pregnancy (which is preventable by vaccine), and certain chromosomal disorders can also increase the risks of truncus arteriosus.

How is Truncus Arteriosis diagnosed during pregnancy?

If your doctor suspects truncus arteriosus after reviewing your routine ultrasound, he or she may request a fetal echocardiogram, or an ultrasound of the fetus’s heart. This echocardiogram shows the structure of the heart and how it is functioning, which can help us confirm the diagnosis and decide on the best option for treatment.

How is Truncus Arteriosus monitored and treated during pregnancy?

After diagnosing Truncus Arteriosus during your pregnancy, our team monitors your baby closely during pregnancy and helps prepare for delivery.

How does Truncus Arteriosus affect delivery?

Most babies with Truncus Arteriosus can be delivered vaginally, unless there are obstetric indications for another mode of delivery. At the Fetal Care Institute, our team of fetal cardiologists and maternal-fetal medicine specialists monitor your baby closely during delivery to minimize complications, and make state-of-the-art care available immediately.

The Fetal Heart Program at the St. Louis Fetal Care Institute has the experience, facilities and technology to handle the most medically challenging deliveries. In the most severe cases the team can perform cesarean sections and EXIT procedures at SSM Cardinal Glennon Children’s Medical Center. The EXIT procedure allows the fetal and cardiothoracic surgeons to perform life-saving procedures on the baby before cutting the umbilical cord. If additional intervention is needed, the baby is next door to the pediatric operating room and the pediatric hybrid cardiac catheterization suite.

What is the surgery for Truncus Arteriosus, and what are the long-term effects?

Most babies with truncus arteriosus need surgery during the first two to three months of life. During surgery, your baby’s surgeon will perform a procedure that artificially constructs the two vessels. The surgeon first patches or sews up the hole between the two ventricles, then implants a tube to connect the right ventricle with the upper portion of the pulmonary artery, and finally reconstructs the aorta. The surgeon may also need to implant a new valve separating the left ventricle and the aorta.

After the surgery, children need regular follow-up visits with a cardiologist to monitor their progress, but will likely lead healthy lives.

Because this tube is artificial, as your child grows, the tube doesn’t grow with him or her—your child will probably need follow-up surgeries. There may be some rigorous athletic activities that your child will not be able to do, but for the most part, he or she will be able to participate fully in most childhood activities. Many truncus arteriosus patients grow up to be healthy adults and have children of their own