Elzenga N J, Gittenberger-de Groot A C
Int J Cardiol. 1985 Aug;8(4):379-93. doi: 10.1016/0167-5273(85)90115-9.
The aortic arches of 34 specimens with hypoplastic left heart syndrome were studied in order to establish the frequency, the nature and the clinical implications of aortic arch anomalies. A localized aortic coarctation was present in 23 specimens. The coarcation was located preductally in 2 and paraductally in 21 cases. The degree of obstruction caused by the coarctation varied considerably. In only 6 cases (1 preductal and 5 paraductal) was the obstruction judged to be severe. One of these cases had an additional aneurysm of the aortic wall proximal to the coarctation. An aberrant relation of the ductus arteriosus and the aortic arch was found in 2 specimens without localized coarctation. In the remaining 9 specimens the aortic arch appeared normal. The aortic arch anomalies were mostly present in specimens with a severely hypoplastic ascending aorta. Clinicians, when preparing infants with hypoplastic left heart syndrome for surgical palliation, should always suspect associated aortic arch anomalies, especially when there is severe hypoplasia of the ascending aorta. Coarctation of the aorta will require additional surgical treatment.
为了确定主动脉弓异常的发生率、性质及临床意义，对34例左心发育不全综合征标本的主动脉弓进行了研究。23例标本存在局限性主动脉缩窄。缩窄位于导管前的有2例，导管旁的有21例。缩窄所致梗阻程度差异很大。仅6例（1例导管前和5例导管旁）梗阻被判定为严重。其中1例在缩窄近端还有主动脉壁动脉瘤。在2例无局限性缩窄的标本中发现动脉导管与主动脉弓关系异常。其余9例标本的主动脉弓外观正常。主动脉弓异常大多出现在升主动脉严重发育不全的标本中。临床医生在为左心发育不全综合征患儿准备手术姑息治疗时，应始终怀疑存在相关的主动脉弓异常，尤其是在升主动脉严重发育不全时。主动脉缩窄需要额外的手术治疗。
