Muraoka Mamoru, Nagata Hazumu, Hirata Yuichiro, Uike Kiyoshi, Terashi Eiko, Morihana Eiji, Ochiai Masayuki, Fujita Yasuyuki, Kato Kiyoko, Yamamura Kenichiro, Ohga Shouichi
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Heart Vessels. 2018 Mar;33(3):309-315. doi: 10.1007/s00380-017-1056-6. Epub 2017 Sep 30.
Right aortic arch with aberrant left subclavian artery (RAA/aLSCA) is a rare aortic arch anomaly. The clinical association of aLSCA stenosis with RAA/aLSCA has not yet been fully elucidated. The aim of this study was to investigate the diagnosis, incidence, management and outcome of aLSCA stenosis in infants with prenatally diagnosed RAA/aLSCA. Ten fetuses who were diagnosed as having RAA/aLSCA in Kyushu University Hospital between January 2011 and December 2014 were enrolled. The maternal and child medical records were reviewed to investigate sex, gestational age at the fetal diagnosis, gestational age and body weight at birth, the findings of computed tomography (CT), Doppler ultrasonography of the vertebral artery and angiography, and the complications and outcomes of aLSCA stenosis. In 8 of 10 patients, aLSCA stenosis was identified on the first CT examination after birth. No patients had dysphagia or respiratory distress. The stenosis spontaneously resolved in 3 patients. In 4 of the 5 remaining patients, aLSCA stenosis progressed, including one case in which complete occlusion occurred-the case was associated with retrograde flow from the left vertebral artery supplying the distal LSCA. Balloon angioplasty was successfully used to treat stenosis in two cases. The subclavian steal phenomenon and developmental problems were not observed in any patients. aLSCA stenosis was identified in 80% of patients with RAA/aLSCA after birth. The early detection and elective treatment of stenotic lesions may be required to prevent complete occlusion during the development of the cardiovascular and cerebrovascular systems.
右位主动脉弓伴迷走左锁骨下动脉（RAA/aLSCA）是一种罕见的主动脉弓异常。aLSCA狭窄与RAA/aLSCA的临床关联尚未完全阐明。本研究的目的是调查产前诊断为RAA/aLSCA的婴儿中aLSCA狭窄的诊断、发病率、管理及结局。纳入2011年1月至2014年12月在九州大学医院诊断为RAA/aLSCA的10例胎儿。回顾母婴医疗记录，以调查性别、胎儿诊断时的孕周、出生时的孕周和体重、计算机断层扫描（CT）结果、椎动脉多普勒超声检查及血管造影结果，以及aLSCA狭窄的并发症和结局。10例患者中有8例在出生后的首次CT检查中发现aLSCA狭窄。无患者出现吞咽困难或呼吸窘迫。3例患者的狭窄自行缓解。其余5例患者中有4例aLSCA狭窄进展，其中1例发生完全闭塞，该病例与来自供应左锁骨下动脉远端的左椎动脉的逆行血流有关。2例患者成功采用球囊血管成形术治疗狭窄。所有患者均未观察到锁骨下动脉窃血现象及发育问题。出生后80%的RAA/aLSCA患者发现有aLSCA狭窄。可能需要早期发现并选择性治疗狭窄病变，以防止在心血管和脑血管系统发育过程中发生完全闭塞。
