Giudici Valentina, Kanani Mazyar, Muthialu Nagarajan, Carr Michelle, Calder Alistair D, Owens Catherine M, Cook Andrew C, Marek Jan
1Great Ormond Street Hospital for Children,London,United Kingdom.
2Institute of Cardiovascular Sciences,University College London,London,United Kingdom.
Cardiol Young. 2016 Feb;26(2):340-6. doi: 10.1017/S1047951115000281. Epub 2015 Mar 5.
We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicated left pulmonary artery with pulmonary sling caused by the retro-tracheal course of the lower left pulmonary artery associated with "Christmas Tree" arrangement of the tracheo-bronchial system. In both patients with pseudo-pulmonary sling and Kabuki syndrome, the abnormal finding was incidental during echocardiographic examination and neither of the patients required surgical repair for the condition. To the best of our knowledge, they represent the third and fourth cases in which such an anomaly of the pulmonary artery branches not forming a sling is seen in association with Kabuki syndrome. Another case represents our second experience and the second case reported in literature with duplicated left pulmonary artery in the setting of a complex tracheal anatomy. In this symptomatic patient, surgical repair of atrial septal defect and relief of the vascular ring were indicated, and the surgical repair was performed successfully at the age of 3 years.
其中两例发生在歌舞伎综合征患儿中,构成了假性肺吊带的情况,且没有任何气管受压的临床或心脏横断面证据。另一例则代表由左下肺动脉经气管后走行并伴有气管支气管系统“圣诞树”样排列导致的左肺动脉重复伴肺吊带。在两例假性肺吊带合并歌舞伎综合征的患者中,异常发现在超声心动图检查时为偶然发现,且两名患者均无需针对该病症进行手术修复。据我们所知,它们是第三和第四例与歌舞伎综合征相关的肺动脉分支未形成吊带的此类异常病例。另一例代表了我们的第二次经验,也是文献中报道的第二例在复杂气管解剖结构背景下左肺动脉重复的病例。在这名有症状的患者中,需要进行房间隔缺损的手术修复和血管环松解,手术修复在3岁时成功完成。