What arises from the truncus arteriosus?

In babies born with truncus arteriosus, the single large vessel never finished dividing into two separate vessels and the wall separating the two ventricles never closed completely, resulting in a single blood vessel arising from the heart, and a large hole between the two chambers (ventricular septal defect).

What is truncus arteriosus?

Truncus arteriosus is a birth defect of the heart. It occurs when the blood vessel coming out of the heart in the developing baby fails to separate completely during development, leaving a connection between the aorta and pulmonary artery.

Is truncus arteriosus genetic?

The exact cause of truncus arteriosus is not known. It has been suggested that some cases may develop due to the interaction of many genetic and environmental factors (multifactorial inheritance). The malformation is the result of an error in embryonic development.

How is truncus arteriosus related to aorta coarctation?

In approximately 1% of cases, truncus arteriosus is associated with severe coarctation of the aorta or interrupted aortic arch (IAA) (Van Praagh type A4). In these cases, the ductus arteriosus is present and is the primary source of blood flow to the lower body. TA with IAA cases are more common than the cases with coarctation of the aorta.

What are the symptoms of coarctation of the aorta?

Patients with a severe coarctation of the aorta are dependent on a patent ductus arteriosus (PDA) to preserve systemic blood flow and perfusion. CoA can be present with an isolated narrowing of the aortic isthmus or with tubular hypoplasia of the aortic arch (transverse arch and isthmus).

How is truncus arteriosus classified as pulmonary atresia?

Truncus Arteriosus. This type is now categorized as a form of pulmonary atresia with a ventricular septal defect rather than TA. The Van Praagh classification system is based on where the branch pulmonary arteries arise from the trunk as well as the development of the aortic arch and the presence of a patent ductus arteriosus (PDA).

How often does truncus arteriosus occur in babies?

Truncus arteriosus (TA) is a rare form of congenital heart disease occurring in 1-3% of patients with congenital heart disease. Truncus arteriosus has an estimated birth incidence of approximately 7 to 21 per 100,000 live births.