完全性大血管错位和右心室双出口肺动脉瓣下室间隔缺损伴主动脉弓病变的一期矫治手术

Single-stage arterial switch operation for transposition of the great arteries and Taussig-Bing with aortic arch obstruction

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 one-stage arterial Switch operation for transposeition of the great arteries （TGA） and Taussig-Bing with aortic arch obstruction. Methods From January 2001 to June 2004, 8 patients had aortic arch obstruction, 3 with TGA and 5 with Taussig-Bing. Except one patient was 8 months old, all of others were 5 clays to 3 months old , the mean operation age was （ 40 ± 36 ） d and the mean weight was （4. 3±0. 5 ） kg. All patients were repaired by one-stage operation. The aortic arch obstruction was repaired in deep hypothermia circulatory arrest, and arterial switch procedure was performed in deep hypothermia and low flow perfusion. Results There had 1 death who was 8 months old and had low cardiac output, complete artrioventricular block （AVB） and severe pulmonary hypertension postoperation. One patient was 3 months old who had asphyxia at 5 clays postoperatively. Six patients followed up from 5 months to 2 years. One Taussig-Bing with interrupted aortic arch had residual obstruction at the anastomosis of aorta. Two had trivial aortic valve regurgitation , and one had mild pulmonary valve regurgitation. Condusions One-stage repair for TGA and Taussig-Bing with aortic obstruction achieves excellent results. The reasons for the death were pulmonary hypertension and abnormal coronary artery. The operative procedure should be performed as early as possible for the better result.