Shahabuddin Syed, Fatimi Saulat, Atiq Mehnaz, Amanullah Muneer
Division of Congenital Cardiac Surgery. Department of Surgery, The Aga Khan University Hospital, Karachi.
J Pak Med Assoc. 2009 Jan;59(1):43-5.
Single ventricle physiology offers limited options with significant morbidity and mortality. The Glenn shunt is a mid-stage procedure for the Fontan circulation. With persistent left Superior Vena Cava (SVC) and Azygos continuation of the Inferior Vena Cava (IVC) to the SVC, needs the development of bilateral bidirectional Glenn shunts. Fifteen patients underwent Bidirectional Glen shunt procedure over a period of ten years. Of these two were found to have left SVC along with interrupted IVC and its Azygos continuation to the SVC. These two patients underwent the Kawashima operation. A seven years old girl was admitted via emergency with severe cyanosis. She had tricuspid atresia, azygos continuation of IVC with single ventricle physiology. She had a complicated postoperative course with prolonged hospital stay after bilateral bidirectional Glen shunt (Kawashima operation). She successfully has completed Fontan after 5 years. The second case ofa 7 year old girl had elective surgery for DORV (double outlet right ventricle), pulmonary atresia and azygos continuation of IVC and persistent left SVC. She had an uneventful postoperative course. The Kawashima operation is an established surgical procedure to deal with bilateral SVCs. The procedure is safe; outcome is favourable and prepares the patient for total cavo-pulmonary shunt.
单心室生理状况提供的治疗选择有限，且发病率和死亡率都很高。格林分流术是用于Fontan循环的中期手术。当存在永存左上腔静脉（SVC）以及下腔静脉（IVC）奇静脉延续至SVC时，则需要进行双侧双向格林分流术。在十年间，有15名患者接受了双向格林分流术。其中两名患者被发现存在左上腔静脉，同时下腔静脉中断且其奇静脉延续至SVC。这两名患者接受了川岛手术。一名七岁女孩因严重紫绀急诊入院。她患有三尖瓣闭锁、下腔静脉奇静脉延续伴单心室生理状况。在接受双侧双向格林分流术（川岛手术）后，她术后病程复杂，住院时间延长。五年后她成功完成了Fontan手术。第二例是一名七岁女孩，因右心室双出口（DORV）、肺动脉闭锁、下腔静脉奇静脉延续以及永存左上腔静脉接受了择期手术。她术后病程平稳。川岛手术是一种既定的处理双侧上腔静脉的外科手术。该手术安全；预后良好，并为患者进行全腔静脉-肺动脉分流术做好准备。
