Truncus arteriosus

Truncus arteriosus (TA) is a rare congenital cardiovascular anomaly characterized by a single arterial trunk arising from the heart by means of a single semilunar valve (<I>i.e.</I> truncal valve). Pulmonary arteries originate from the common arterial trunk distal to the coronary arteries and proximal to the first brachiocephalic branch of the aortic arch. TA typically overrides a large outlet ventricular septal defect (VSD). The intracardiac anatomy usually displays situs solitus and atrioventricular (AV) concordance.

Hernia



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PMID (PMCID)
10602117
FEMALE Infant, Newborn
Truncus arteriosus and other lethal internal anomalies in Goltz syndrome.
Han XY, Wu SS, Conway DH, Pawel BR, Punnett HH, Martin RA, de Chadarevian JP.
Am J Med Genet. 2000;90(1):45-8.
The internal anomalies included truncus arteriosus type II with truncal origin of hypoplastic pulmonary arteries, cardiac ventricular septal defect, severe hypoplasia of lungs and pulmonary veins, massive diaphragmatic hernia, and absence of the right kidney.
8831133
MALE Infant, Newborn
Partial trisomy and partial monosomy of the distal long arm of chromosome 4: patient report and literature review.
Frints SG, Schrander-Stumpel CT, Engelen JJ, Da Costa AJ, Fryns JP.
Genet Couns. 1996;7(2):135-42.
The boy died from a complex cardiac defect (monoventricle, monoatrium and truncus arteriosus) in combination with a diaphragmatic hernia.