What is Truncus Arteriosus?

When a person has one large artery instead of two separate ones to carry blood to the lungs and body.

In a normal heart, the blood follow this cycle: body-heart-lungs-heart-body. When a person has a truncus arteriosus, the blood leaving the heart does not follow this path. It has only one vessel, instead of two separate ones for the lungs and body. With only one artery, there is no specific path to the lungs for oxygen before returning to the heart to deliver oxygen to the body.

Can it be repaired?

Surgery is necessary to close the ventricular septal defect and separate blood flow to the body from blood flow to the lungs. This is usually done early in infancy to prevent high blood pressure from damaging the lung arteries. A patch is used to close the ventricular defect. The pulmonary arteries are then disconnected from the single great vessel (the truncus) and a tube (a conduit or tunnel) is placed from the right ventricle to the pulmonary arteries. This is sometimes called a Rastelli repair.

Will my child need more surgery?

The conduit connecting the right ventricle to the pulmonary artery conduit may become narrowed and blocked (stenotic) over time, or the child may outgrow the conduit. It may have to be replaced from time to time. Timing of the replacement varies. The peripheral pulmonary arteries also may become narrowed and require treatment. Sometimes conduits and peripheral pulmonary artery narrowings may be dilated using a balloon-tipped catheter or an expandable stent in the cardiac catheterization laboratory. This procedure may help extend the time between operations for conduit changes. Sometimes surgery is required to enlarge the narrowed area. Your child's cardiologist will discuss whether a balloon/stent procedure or surgery is best.

The aortic valve is actually the large truncal valve from the single vessel, which arose over the ventricular septal defect before surgical repair. This valve sometimes becomes leaky over time and may need to be replaced.

What ongoing care will my child need?

Children with truncus arteriosus need regular follow-up with a pediatric cardiologist and they may need to take medicine after surgery. Your child's cardiologist will evaluate with a variety of tests including electrocardiograms and echocardiograms to determine when another procedure such as cardiac catheterization may be needed.

*Information provided by www.heart.org