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目的探讨主动脉缩窄和(或)主动脉弓发育不良并心内畸形的手术治疗。方法选择2013年1月—2014年6月收治的41例主动脉缩窄和(或)主动脉弓发育不良患儿作为研究对象,根据术式分为选择性顺行脑灌注(selective antegrade cerebral perfusion,SACP)组11例和深低温停循环(deep hypothermic circulatory arrest,DHCA)组30例,记录并比较两组患儿主动脉阻断时间、体外循环时间、呼吸机辅助时间、监护室时间、住院天数及患儿随访情况。计量资料采用t检验,P<0.05为差异有统计学意义。结果 SACP组主动脉阻断时间、体外循环时间、住院时间分别为(63.57±9.66)、(107.82±12.28)min、(14.95±3.06)d,与DHCA组的(50.84±6.93)、(91.46±11.43)min、(18.61±3.07)d比较差异均有统计学意义(均P<0.05)。1名患儿术中出现严重低心排出量不能脱离体外循环而导致死亡;术后死于低心排综合征1例,死于肺部感染1例,死于突发恶性心律失常1例;4例患儿术后出现声嘶,随访2个月后均基本恢复。术后随访中发现吻合口缩窄1例,吻合口成角扭曲1例;存活患儿均无明显神经系统并发症、肺动脉高压危象、脊髓损伤,脑电图异常发生。结论主动脉缩窄和(或)主动脉弓发育不良并心内畸形患者应该积极就医治疗,术中应酌情选择SACP或DHCA技术,扩大端-端吻合术和端-侧吻合术临床疗效较好。
Objective To investigate the surgical treatment of aortic constriction and / or aortic arch dysplasia and intracardiac deformity. Methods From January 2013 to June 2014,41 children with aortic constriction and / or aortic arch dysplasia were enrolled and divided into selective antegrade cerebral perfusion (SACP) ) Group and 30 patients with deep hypothermic circulatory arrest (DHCA) group. The aorta occlusion time, cardiopulmonary bypass time, ventilator support time, care room time, hospitalization days, Follow-up of children. Measurement data using t test, P <0.05 for the difference was statistically significant. Results The time of aortic block, cardiopulmonary bypass and hospital stay in SACP group were (63.57 ± 9.66), (107.82 ± 12.28) min and (14.95 ± 3.06) d, respectively, which were significantly higher than those in DHCA group (50.84 ± 6.93 and 91.46 ± 11.43) min, (18.61 ± 3.07) d respectively. There were significant differences between the two groups (all P <0.05). One child with severe low cardiac output during operation could not be separated from cardiopulmonary bypass and died; one died of low cardiac output syndrome after operation, one died of pulmonary infection and one died of sudden malignant arrhythmia. 4 cases of children with hoarseness after surgery, were followed up for 2 months after the basic recovery. One case of anastomotic stenosis and 1 case of anastomotic angulation were found in the follow-up. There were no obvious neurological complications, pulmonary hypertension risk, spinal cord injury and EEG abnormality in surviving children. Conclusions Patients with aortic constriction and / or aortic arch dysplasia and intracardiac deformity should seek active medical treatment. SACP or DHCA should be selected as appropriate during operation. The clinical efficacy of expanding end-to-side anastomosis and end-to-side anastomosis is good.