Rey C, Coeurderoy A, Dupuis C
Arch Mal Coeur Vaiss. 1984 May;77(5):526-33.
Two cases of the association of Fallot's tetralogy and coarctation of the aorta are reported. In both cases there was a right aortic arch. In addition, the second case had a cervical aorta and a left subclavian artery isolated from the aorta and vascularised by the left vertebral artery. The two children underwent complete repair: the first patient had a Waterston shunt followed by repair of the Fallot's tetralogy, followed by repair of the coarctation; the second patient first had a Gore-tex tube inserted from the ascending to the descending aorta and then underwent repair of the Fallot's tetralogy. The association of a coarctation with an obstructive lesion of the right heart is very rare; only two cases have previously been reported: one Fallot's tetralogy and one tricuspid atresia with pulmonary stenosis. Both cases also had a right aortic arch. This seems to go against the haemodynamic theory of coarctation: blood flow in the ascending aorta increases while flow in the pulmonary artery decreases. Coarctation could be caused by a partial involution of the terminal segment of the right primitive dorsal aorta as proposed in cases of coarctation with a right sided aortic arch. The second case also presented two uncommon features: a cervical aortic arch and a left subclavian artery isolated from the aorta.
本文报告了两例法洛四联症与主动脉缩窄并存的病例。两例均为右位主动脉弓。此外，第二例患者有颈段主动脉，其左锁骨下动脉与主动脉分离，并由左椎动脉供血。两名患儿均接受了根治性修复：第一例患者先进行了Waterston分流术，随后修复法洛四联症，最后修复主动脉缩窄；第二例患者先在升主动脉和降主动脉之间植入了一段戈尔泰克斯人工血管，然后进行法洛四联症修复。主动脉缩窄与右心梗阻性病变并存的情况非常罕见；此前仅报道过两例：一例为法洛四联症，另一例为三尖瓣闭锁合并肺动脉狭窄。这两例也均为右位主动脉弓。这似乎与主动脉缩窄的血流动力学理论相悖：升主动脉血流增加而肺动脉血流减少。主动脉缩窄可能如右位主动脉弓型主动脉缩窄病例中所提出的那样，是由于右原始背主动脉终末段的部分退化所致。第二例还呈现出两个罕见特征：颈段主动脉弓和与主动脉分离的左锁骨下动脉。
