Truncus Arteriosus Repair

Overview of Truncus Arteriosus Repair in India

During the development of the fetus, there is typically one large vessel (the truncus arteriosus) that exits the heart. When this does not happen at the time of birth, it is termed truncus arteriosus, a rare type of congenital heart defect. In this condition, only one major blood vessel exits the heart (the truncus arteriosus) as opposed to having two (the pulmonary artery from the right ventricle and the aorta from the left).

In premature infants, the truncus arteriosus fails to separate into the two distinct vessels that are normally formed from the right and left ventricles. This leaves a large hole in the septum (the partition) of the right and left ventricles. The hole allows excessive blood flow into the pulmonary arteries (lungs) because the right ventricle has to work harder to pump blood to both the lungs and the body. This condition is life-threatening and must be repaired surgically soon after delivery.

What Is Truncus Arteriosus?

Truncus arteriosus, sometimes called a common arterial trunk, is a rare heart defect that some babies are born with. Instead of having two separate arteries carrying blood out of the heart, there’s just one. Only one valve—the truncal valve—manages all the blood flow.

Here’s what usually happens: the pulmonary artery takes oxygen-poor blood to the lungs, and the aorta sends oxygen-rich blood out to the body. But with truncus arteriosus, that separation disappears. One big artery leaves the heart, so oxygen-rich and oxygen-poor blood mix together.

This really strains the heart and lungs, and the body doesn’t get enough oxygen. That’s why doctors call truncus arteriosus a critical congenital heart defect. Babies with this condition need surgery soon after birth to fix the problem and restore normal blood flow.

It’s rare—only about 1 in every 16,000 babies born in the U.S. has truncus arteriosus. Compared to other heart defects, it doesn’t happen often. Out of all babies diagnosed with a heart condition, just 2% have this one.

Types of Truncus Arteriosus Repair:

Truncus arteriosus comes in four types, but they all have one thing in common: there’s just a single large vessel leaving the heart. What sets them apart is where the right and left pulmonary arteries split off and head toward each lung. This detail matters because it changes how doctors fix the problem.

Type 1 is the one doctors see most often—about 60% of cases. Here, both pulmonary arteries branch off from a main pulmonary artery, which itself comes off the common trunk.

With type 2, the right and left pulmonary arteries each branch straight from the common trunk. No main pulmonary artery in between.

Type 3 looks a bit different. One pulmonary artery comes off the common trunk, while the other takes off from the aorta instead.

Then there’s type 4, the rarest one. In this case, neither pulmonary artery branches from the main trunk at all. Instead, they both come off the descending aorta—the part that runs down through the chest.

Procedure:

Usually, the only way to treat truncus arteriosus is through surgery. However, the doctor might recommend the following course of action based on the baby's condition:

Surgery

Truncus arteriosus causes many babies to need surgery within weeks of being born. The baby undergoes an open-heart operation through the chest. The operative procedure can involve incisions on the chest wall and applying a patch to cover holes in the heart muscle. The pulmonary arteries will be separated from the truncus and connected to the right ventricle of the heart with surgical tubing made for that purpose.

During the same operation, the surgeon meets the requirements of reconstructing one main artery and creating a new main artery (aorta) that will connect with the left ventricle. The baby must continue seeing a cardiologist for life after surgical repair to monitor for complications. The baby may be placed on antibiotics or other medications postoperatively. If the baby grows significantly during the course of childhood, it might be necessary to have two or three additional operations to replace the tubing from the first operation.

Medications

Medications required for your child can include:

Diuretics - this type of medication helps to prevent too much fluid from building in the body, and thus helps to increase the amount of urine produced.

ACE Inhibitors - these types of medications can lower blood pressure and also assist the heart with its ability to pump blood more effectively.

Decongestants - these medications will help to strengthen the heart muscle itself.