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Persistent truncus arteriosus (PTA), [1] often referred to simply as truncus arteriosus, [2] is a rare form of congenital heart disease that presents at birth. In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta .
The truncus arteriosus and bulbus cordis are divided by the aorticopulmonary septum.The truncus arteriosus gives rise to the ascending aorta and the pulmonary trunk.The caudal end of the bulbus cordis gives rise to the smooth parts (outflow tract) of the left and right ventricles (aortic vestibule & conus arteriosus respectively). [2]
TPVR can be used to repair congenital defects in the pulmonary valve or right ventricular outflow tract dysfunction, such as pulmonary atresia, Tetralogy of Fallot, or persistent truncus arteriosus. [4] TPVR can also be used to replace dysfunctional artificial heart valves. [1]
The main outflow tract is divided in two by the growth of a spiraling septum, becoming the great vessels—the ascending segment of the aorta and the pulmonary trunk. If the separation is incomplete, the result is a "persistent truncus arteriosus". The vessels may be reversed ("transposition of the great vessels"). The two halves of the split ...
The severity of symptoms depends on the type of TGV, and the type and size of other heart defects that may be present (ventricular septal defect, atrial septal defect, or patent ductus arteriosus). Most babies with TGA have blue skin color (cyanosis) in the first hours or days of their lives, since dextro-TGA is the more common type.
The removal of the cardiac neural crest complex causes a reduction in contractility of the myocardium. In embryos containing persistent truncus arteriosus, there is a significant 2-fold reduction in calcium currents, thereby interrupting the cardiac excitation-contraction coupling process to cause a reduction in contractility. [9] [21]
Persistent truncus arteriosus (minimal cyanosis) Transposition of great vessels; Tricuspid atresia; Tetralogy of Fallot; Total anomalous pulmonary venous return; A mnemonic to remember the conditions associated with right-to-left shunting involves the numbers 1-5, as follows: 1 Combination Vessel: Persistent truncus arteriosus (minimal cyanosis)
The early bulbus cordis is formed by the fifth week of development. [4] The truncus arteriosus is derived from it later. [2]The adjacent walls of the bulbus cordis and ventricle approximate, fuse, and finally disappear, and the bulbus cordis now communicates freely with the right ventricle, while the junction of the bulbus with the truncus arteriosus is brought directly ventral to and applied ...