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Truncus arteriosus

Authors
Brian Soriano, MD
David R Fulton, MD
Section Editor
John K Triedman, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Truncus arteriosus (TA), also known as common arterial trunk, is a cyanotic congenital heart defect. In this condition, blood is pumped from the heart through a single truncal valve into a truncal artery, which gives rise to the aorta and the pulmonary arteries (figure 1).

The anatomy, clinical manifestations, diagnosis, and management of TA will be reviewed here.

EPIDEMIOLOGY

The reported incidence of TA ranges from 6 to 10 per 100,000 live births [1,2]. Although only 0.7 percent of all congenital heart lesions are due to TA, it accounts for 4 percent of all critical congenital heart disease cases [1].

EMBRYOLOGY

During the early stages of a normally developing heart, the great arteries originate from a single truncal root. A wall forms within this root (truncoconal septum) that divides the root into a fully developed main pulmonary artery and ascending aorta by the end of the fifth week of gestation. Over the next two weeks of development, there is closure of the conal (infundibular) septum as the truncoconal septum fuses with the endocardial cushions and the interventricular septum, which separates the newly formed right and left ventricle.

Disturbances in the development of the truncoconal septum result in conotruncal abnormalities including TA. The lack of wall development also impairs the creation of separate aortic and pulmonary valves, resulting in the single truncal valve associated with TA [3].

                                       

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Literature review current through: Nov 2016. | This topic last updated: Tue Jul 12 00:00:00 GMT 2016.
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