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目的总结婴幼儿一侧肺动脉起源异常的外科治疗经验。方法回顾性分析2005年3月至2010年5月第四军医大学西京医院11例一侧肺动脉起源异常合并心血管畸形患者行外科手术治疗的临床资料,手术时平均年龄11.5(2～36)个月;平均体重7.1(4～13)kg。右肺动脉起源于升主动脉7例,左肺动脉起源于升主动脉4例。11例患者均合并其它心血管畸形及中度以上肺动脉高压。全组患者均在低温体外循环下经胸骨正中切口径路行一期手术矫治。结果手术时间169～293(231±55)min,体外循环时间87～210(138±47)min,主动脉阻断时间45～133(86±28)min。围术期死亡1例,病死率9.1%,为法洛四联症合并右肺动脉起源异常患者术后死于低心排血量。生存患者术后超声心动图提示:左、右肺动脉均经右心室肺动脉发出,无狭窄、残余漏,畸形矫治满意。随访10例,随访率100%,平均随访13.5(3～32)个月。超声心动图提示:肺动脉连接均通畅,9例患者的肺动脉压均明显下降。结论一侧肺动脉起源异常应尽早外科手术治疗,婴幼儿一侧肺动脉起源异常行外科手术治疗可获得良好的近期疗效,年龄较大肺动脉压力不可逆增高者外科治疗应谨慎;术中充分游离异常起源的肺动脉及升主动脉,并视具体情况横断主动脉可获得较好的手术显露;自体肺动脉补片加宽修补主动脉缺损可有效避免术后动脉瘤的发生。
Objective To summarize the experience of surgical treatment of the anomalous origin of pulmonary artery in infants and children. Methods The clinical data of 11 patients with abnormal pulmonary artery origin and cardiovascular malformations who were treated in Xijing Hospital of the Fourth Military Medical University from March 2005 to May 2010 were retrospectively analyzed. The average age of the patients was 11.5 (range 2-36) Month; average body weight 7.1 (4 ~ 13) kg. Right pulmonary artery originated in 7 cases of ascending aorta, left pulmonary artery originated in 4 cases of ascending aorta. Eleven patients had combined with other cardiovascular malformations and moderate pulmonary hypertension. All patients underwent hypothermic cardiopulmonary bypass through the sternotomy incision pathological surgery. Results The operative time ranged from 169 to 293 (231 ± 55) min, with an extracorporeal circulation of 87 to 210 (138 ± 47) min and aortic blockade of 45 to 133 (86 ± 28) min. Perioperative death in 1 case, the case fatality rate of 9.1%, for the tetralogy of Fallot with right ventricular dysplasia patients died of low cardiac output. Postoperative echocardiography in patients with tips: left and right pulmonary artery were issued by the right ventricular pulmonary artery, no stenosis, residual leakage, deformity correction satisfied. Follow-up of 10 cases, follow-up rate of 100%, the average follow-up of 13.5 (3 to 32) months. Echocardiography prompted: pulmonary artery connections were smooth, pulmonary artery pressure in 9 patients were significantly decreased. Conclusion One side of the pulmonary artery should be as early as possible surgical treatment of surgical anomalies, infants and young children on the side of the anomalies of pulmonary artery surgery can obtain good short-term effect, older pulmonary artery pressure irreversibly increased surgical treatment should be cautious; surgery full of free anomalous origin Pulmonary artery and ascending aorta, and depending on the specific circumstances of the aorta can be better surgery revealed; autologous pulmonary artery patch to widen repair of aortic defects can effectively prevent the occurrence of postoperative aneurysms.