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Virtual Pediatric Hospital: Paediapaedia: Truncus Arteriosus Paediapaedia: Cardiovascular Diseases

Truncus Arteriosus

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Presents in infancy with congestive heart failure due to left heart volume overload. The patient has mild cyanosis due to a combination of ventricular admixture of systemic and pulmonary venous blood and pulmonary edema. The cyanosis becomes worse as pulmonary hypertension develops.

Etiology/Pathophysiology:
Due to a failure of division of the embryonic truncus arteriosus into the aorta and pulmonary artery. A single great artery leaves the heart and gives rise to the coronary, pulmonary, and systemic arterial circulation. The truncus stradles a high VSD and receives outflow from both ventricles. A truncal valve has 2-6 cusps, and usually is incompetent. Truncus is classified by the origin of the pulmonary artery:

Pathology:
Not applicable

Imaging Findings:
For Type I truncus and Type II and Type III without pulmonary stenosis, increased pulmonary vasculature and cardiomegaly is seen due to biventricular and left atrial enlargement due to the left to right shunt and insufficiency of the truncus valve. It can resemble TGA on CXR, but note that 30% of truncus have a right arch while only 5% of TGA do.

DDX:

References:
See References Chapter.

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