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目的评估一期纠治完全性大血管错位(TGA)和右心室双出口肺动脉瓣下室间隔缺损(Taussig-B ing)伴主动脉弓病变的手术疗效。方法2001年1月—2004年6月对8例伴主动脉弓病变的TGA(3例)和Taussig-B ing(5例)行一期手术治疗。3例TGA中,室间隔完整型1例,伴室间隔缺损2例;主动脉弓病变为7例主动脉缩窄、1例主动脉弓中断。手术年龄1例为8个月,7例为5 d~3个月,平均40 d,体重3.5~6.3 kg,平均(4.3±0.5)kg。均采用胸骨正中切口。手术先在深低温、停循环下矫治主动脉弓病变,然后在深低温、低流量下行大动脉转换术(Sw itch术)。体外循环转流时间107~159 m in,平均(126±23)m in,主动脉阻断时间63~118 m in,平均(92±16)m in,停循环14~45 m in,平均(30±12)m in。结果手术死亡1例,为8个月Taussig-B ing伴主动脉弓发育不良、冠状动脉畸形患儿,术后因低心排血综合征、Ⅲ度房室传导阻滞、肺高压危象死亡;1例3月龄患儿术后5 d喂奶时窒息死亡。6例随访5个月~2年,生长发育良好,1例Taussig-B ing主动脉弓中断出现吻合口狭窄,压差60 mm Hg;2例出现主动脉瓣轻微返流,1例肺动脉瓣轻度返流。结论一期纠治TGA和Taussig-B ing伴主动脉弓病变能取得较好手术效果,手术死亡原因为肺动脉高压和冠状动脉畸形。
Objective To evaluate the efficacy of primary correction of TGA and Taussig-Bing with aortic arch lesion in double outlet right ventricle. Methods TGA (three cases) and Taussig-B ing (eight cases) with aortic arch lesion underwent primary surgery from January 2001 to June 2004. One patient had ventricular septal defect and two patients had ventricular septal defect in 3 TGA patients. Aortic arch lesion was narrowed in 7 patients and aortic arch was interrupted in 1 patient. One patient was operated on for 8 months and 7 patients for 5 days to 3 months with an average of 40 days and 3.5 to 6.3 kg (average 4.3 ± 0.5) kg. Sternal midline incision are used. The procedure is to first treat the aortic arch lesion under deep hypothermia and stop circulatory arrest, and then perform the Switch procedure in deep hypothermia and low flow. The time of extracorporeal circulation was 107 ~ 159 mins (average, 126 ± 23) mins, the aortic block time was 63 ~ 118 mins, mean (92 ± 16) mins, 30 ± 12) m in. Results One patient died of Taussig-B ing with aortic arch dysplasia in 8 months. The children with coronary artery malformations died of low cardiac output syndrome, grade Ⅲ atrioventricular block and pulmonary hypertension. Example 3-month-old children died of suffocation after 5 days of operation. 6 cases were followed up for 5 months to 2 years, with good growth and development. One case of anastomosis of Taussig-B ing aortic arch showed anastomotic stenosis with a pressure difference of 60 mm Hg; 2 cases had mild aortic regurgitation and 1 case mild pulmonary valve retrograde flow. Conclusion The first-stage correction of TGA and Taussig-B ing with aortic arch lesions can achieve better surgical results. The causes of death from surgery are pulmonary hypertension and coronary artery malformations.