Honey M
Q J Med. 1977 Oct;46(184):463-83.
Six new cases of the "scimitar syndrome" are described. The anatomical and haemodynamic features of these and other reported cases are reviewed. Within the spectrum of the disorder there is a group of patients in whom the bronchopulmonary manifestations are relatively unimportant. In five of our patients there was a left-to-right shunt exceeding 2:1 and the anomalous pulmonary venous connection was corrected surgically. The presence or absence of an associated atrial septal defect may be difficult to establish but influences the choice of surgical technique. When the atrial septum is intact, the anomalous vein should be reimplanted if possible into the back of the left atrium; otherwise a pericardial or teflon patch can be used to redirect the anomalous venous return through an existing or created atrial septal defect to the left atrium.
本文描述了6例新的“弯刀综合征”病例。对这些病例以及其他已报道病例的解剖学和血液动力学特征进行了回顾。在该疾病范围内,有一组患者,其支气管肺表现相对不严重。我们的5例患者存在超过2:1的左向右分流,异常肺静脉连接通过手术得到纠正。是否存在相关房间隔缺损可能难以确定,但会影响手术技术的选择。当房间隔完整时,如有可能,应将异常静脉重新植入左心房后壁;否则,可使用心包或聚四氟乙烯补片,通过现有的或制造的房间隔缺损将异常静脉回流改道至左心房。