Abstract：
Aberrant subclavian artery (ASCA) is frequently observed in interrupted aortic arch (IAA) with aortic/subaortic obstruction. Developmental significance of ASCA in IAA in utero remains elusive. Newborns with prenatally diagnosed isolated IAA under continuous prostaglandin E1 infusion were studied. Cross-sectional areas of aortic valve opening (AVOCSA) and patent ductus arteriosus (PDACSA) were represented by echocardiographic measurement of (diameter)2 indexed by body surface area (m2). Types of IAA and presence of ASCA were examined in relation to sizes of AVOCSA and PDACSA. Twenty-four newborns with IAA (six type A and 18 type B) were reviewed. Male dominance was seen in type B (male 72%). Twenty-three patients had left aortic arch. No type A patients had ASCA, but 50% of type B had ASCA; AVOCSA was significantly smaller in type B than in type A (p = 0.003). In type B, PDACSA was significantly larger in those with ASCA than without (p = 0.003), but AVOCSA exhibited no significant size difference between these two subgroups. Chromosome 22q11 deletion was only seen in type B (56%) and showed no significant correlation with the presence of ASCA. In type B IAA, the presence of ASCA was associated with larger PDACSA, suggesting an adaptive enlargement of the ductus arteriosus and ASCA in response to reduced antegrade flow across small AVOCSA, which may be augmenting cerebral blood flow. Preservation of cerebral blood flow may be another important determinant affecting embryonic cardiovascular development.
摘要：
在主动脉弓（IAA）中断并伴有主动脉/主动脉下阻塞的主动脉弓（IAA）中经常观察到异常的锁骨下动脉（ASCA）。子宫内IAA中ASCA的发育意义仍然难以捉摸。研究了连续输注前列腺素E1的产前诊断为IAA的新生儿。主动脉瓣开口（AVOCSA）和动脉导管未闭（PDACSA）的横截面面积通过超声心动图测量（直径）2以体表面积（m2）为索引。根据AVOCSA和PDACSA的大小检查了IAA的类型和ASCA的存在。回顾了24例IAA新生儿（6例A型和18例B型）。在B型中可见男性优势（男性72％）。23例患者患有左主动脉弓。没有A型患者有ASCA,但50%的B型患者有ASCA;B型AVOCSA明显小于A型(p=0.003).在B型中，PDACSA在有ASCA的患者中明显大于没有ASCA的患者（p=0.003），但是AVOCSA在这两个亚组之间没有显着的大小差异。染色体22q11缺失仅在B型（56％）中可见，与ASCA的存在没有显着相关性。在B型IAA中，ASCA的存在与较大的PDACSA有关，提示动脉导管和ASCA的适应性扩大，以响应小AVOCSA的顺行流量减少，这可能会增加脑血流量。脑血流量的保持可能是影响胚胎心血管发育的另一个重要决定因素。
