Pulmonary Atresia and Ventricular Septal Defect

A review of the options for treatment of major aortopulmonary collateral arteries in the setting of tetralogy of Fallot with pulmonary atresia

“…These stenoses may progressively worsen due to ongoing turbulent flow, 213 polycythemia, hyperviscosity, and lack of growth. 13 Due to mounds of intimal tissue containing significant amounts of collagen, the stenoses may become tough and resistant to extreme high forces beyond 20 atmospheres.…”

Stenting of stenosed aortopulmonary collaterals and shunts for palliation of pulmonary atresia/ventricular septal defect

“…APCAs are embryologically programmed not to survive beyond early fetal life and should they persist, there is a 58%-68% incidence of significant stenosis [6,7]. The stenosis is caused by mounds of intimal tissue that protrude into the lumen and further progresses due to turbulent flow, polycythemia, hyperviscosity, and lack of growth [7][8][9][10].…”

Aortopulmonary collateral arteries in a child with trisomy 21

“…Congenital aortopulmonary collateral arteries are a common finding in tetralogy of Fallot with pulmonary atresia, and have also been described in other forms of congenital cardiac disease, as well as being recognized as an incidental finding during transcatheter occlusion of persistent patency of the arterial duct. 1,6 Isolated and truly congenital aortopulmonary collateral arteries, however, are very rare without associated cardiopulmonary malformations or prematurity, and have so far not been demonstrated to cause clinical symptoms.…”

“…A ORTOPULMONARY COLLATERAL ARTERIES ARE a well-recognized finding in congenital cardiac disease, most frequently associated with tetralogy of Fallot with pulmonary atresia. 1 They have also been described in association with several different acquired and congenital bronchopulmonary disorders. 2 Although isolated aortopulmonary collateral arteries are rare in infants with normal respiratory function, recent studies suggest that they are a common finding in infants born with very low weight, especially those requiring long periods of positive pressure ventilation with secondary bronchopulmonary dysplasia.…”