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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 .
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.
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]
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 ...
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.
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.
Prognosis is dependent upon the health of the child, as there is an increased demand on respiratory and heart rate in infants during common childhood illnesses. This fragile population has little cardiac reserve to accommodate these demands and provide hemodynamic stability during illnesses.
The aortic sac or aortic bulb [1] is a dilated structure in mammalian embryos, lined by endothelial cells and is the most distal part of the truncus arteriosus. [2] It is the primordial vascular channel from which the aortic arches arise (and eventually the dorsal aortae) and is homologous to the ventral aorta of gill-bearing vertebrates.