Uematsu M, Yamaguchi M, Ohashi H, Imai M, Oshima Y, Ataka K, Yoshimura N
Department of Cardio-Thoracic Surgery, Kobe Children's Hospital, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1994 Aug;42(8):1247-53.
A 33-day-old girl was transferred to our hospital because of severe cyanosis and tachypnea. The diagnosis of aortopulmonary window and interrupted aortic arch was established by echocardiogram and cineangiocardiogram. Reconstruction of aortic arch by extended direct anastomosis and simple patch closure of aortopulmonary window were performed by means of profound hypothermia and circulatory arrest on 36 days of age. Her postoperative clinical course was uneventful, but at 2 months after operation, stenosis of the ascending aorta was noticed by echocardiographic examination. As her physical growth had been satisfactory, she was managed under close observation at outpatient clinic. At seven months after operations, she fell into progressive left heart failure, and emergency operation was carried out for the relief of stenosis of the ascending aorta, but she could not come off bypass. A stenosis of the ascending aorta is a fatal complication that may occur after one-stage repair of interrupted aortic arch with aortopulmonary window consisting of extended direct anastomosis and simple patch closure. We now consider that division of ascending aorta and pulmonary artery and repair of both of the defect is indispensable for the correction of aortopulmonary window, especially in neonate and small infant with this lesion associated with interrupted aortic arch.
一名33日龄女婴因严重发绀和呼吸急促被转诊至我院。通过超声心动图和心血管造影确诊为主肺动脉窗和主动脉弓中断。在患儿36日龄时，采用深低温停循环技术，通过扩大直接吻合重建主动脉弓，并简单修补主肺动脉窗。术后临床过程平稳，但术后2个月超声心动图检查发现升主动脉狭窄。由于其身体生长发育良好，故在门诊密切观察下进行处理。术后7个月，患儿出现进行性左心衰竭，遂急诊手术解除升主动脉狭窄，但未能脱离体外循环。升主动脉狭窄是一种致命的并发症，可能发生在一期修复主动脉弓中断合并主肺动脉窗（采用扩大直接吻合和简单修补）之后。我们现在认为，对于主肺动脉窗的矫治，尤其是合并主动脉弓中断的新生儿和小婴儿，切断升主动脉和肺动脉并修复两者的缺损是必不可少的。
