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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]
Tetralogy of Fallot with pulmonary atresia (pseudotruncus arteriosus) is a severe variant [47] in which there is complete obstruction (atresia) of the right ventricular outflow tract, causing an absence of the pulmonary trunk during embryonic development.
Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. There are three types of interrupted aortic arch, with type B being the most common. Interrupted aortic arch (especially Type B) is often associated with DiGeorge syndrome.
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 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 truncus arteriosus will divide to form the aorta and pulmonary artery; the bulbus cordis will develop into the right ventricle; the primitive ventricle will form the left ventricle; the primitive atrium will become the front parts of the left and right atria and their appendages, and the sinus venosus will develop into the posterior part of ...