How Could Aortic Atresia With Interrupted Aortic Arch Survive? About a Neonatal Repair on Two Ventricles

Aortic valve atresia and type C interrupted aortic arch with circle of Willis dependent circulation: a case of successful biventricular repair in the neonatal period

“…Aortic valve atresia and interrupted aortic arch are rare combination with unique considerations regarding cerebral and myocardial perfusion. This anatomy with a type C interruption has only been described three other times in the literature, [1][2][3][4] with only one case involving circle of Willis dependent circulation. 3 The other two cases involved the presence of bilateral ductus.…”

“…1,4 There have been a greater number of reported cases of aortic atresia with type B interruption with wider variety in cerebral and myocardial perfusion, including aberrant right subclavian artery and circle of Willis dependent circulation; bilateral ducti; aortopulmonary window; double aortic arch; fistulous connection from the pulmonary artery to the left coronary artery. 2 The aforementioned case reports are increasingly describing complete neonatal repair, including the case of the type C interruption presented by Malankar et al 3 While similar in our approach with a Yasui and interrupted arch repair, I would like to highlight our unique arterial cannulation approach which avoided circulatory arrest by having two arterial cannulae which allowed for preserved cerebral perfusion via the right subclavian arterial cannula. In all, this was an interesting case of rare anatomy with successful complete biventricular repair with Yasui and type C interrupted aortic arch repair via median sternotomy.…”

Aortic atresia with interrupted aortic arch and bilateral arterial ductus: a successful initial palliation

“…In the majority of the reported cases, perfusion to the brain and myocardium is provided by bilateral ductus, AP window or double arch. 3 Only very few cases had no direct connection to the ascending aorta and all had an aberrant right subclavian artery. 5 In our case, cerebral and coronary perfusion was maintained through a right-sided arterial ductus.…”

“…Different reported sources of blood supply to the blind ascending aortic segment are (1) aorto-pulmonary (AP) window, (2) double aortic arch, (3) bilateral arterial ductus, and (4) aberrant right subclavian artery. [1][2][3] When there are no above-mentioned sources of blood supply is available, it is through the circle of Willis, the blood flow to the brain and myocardium is maintained in a retrograde manner through carotids ascending aorta and then to the coronary arteries. 4 In the absence of an associated anomaly to perfuse the blind segment, type A interrupted aortic arch with aortic atresia is not compatible with life.…”