Truncus Arteriosus

Congenital (meaning from birth) heart defects (CHD) are among the most common defects facing newborn babies, at about 9 out of 1000 live births. Although there are many types of CHD’s, Truncus Arteriosus is one of the most rare, and affects less than 1 out of 10,000 live births. It is considered one of 7 critical heart defects, and is fatal within weeks if not found immediately.

Normally there are two main blood vessels leaving the heart: the aorta carrying oxygen-rich blood to the body and the pulmonary artery that branches immediately to carry oxygen-poor blood to each lung.

Instead of having a separate pulmonary artery and aorta, each with their own three-leafed valves, a baby with truncus arteriosus has only one great blood vessel or trunk leaving the heart, which then branches into blood vessels that go to the lungs and the body.

This great vessel usually has one large valve which may have between two and five leaflets. Usually this great vessel sits over both the left and right ventricle. The upper portion of the wall between these two chambers is missing resulting in what is known as a ventricular septal defect (VSD).As a result of truncus arteriosus, oxygen-poor blood that should go to the lungs and oxygen-rich blood that should go to the rest of the body are mixed together.

Normaltruncus_1_1

Truncustruncus_1_2 Truncus Repairedtruncus_2_2

Truncus can be repaired with an open heart surgery, where the aorta and pulmonary artery are separated and recreated using an implanted conduit made of a gor-tex fabric, or donated human tissue (homograft). As the heart grows, this conduit does not grow with it, and needs to be upsized and replaced via additional open-heart surgeries as the child grows.

 

Judah had his first repair when he was 9 days old, and his second when he was 6 months old.