先天性心脏病合并右位主动脉弓的诊断与治疗

目的 探讨先天性心脏病合并右位主动脉弓的诊断与治疗。 方法 回顾性分析2012年1月1日至2013年4月1日北京儿童医院小儿心脏中心27例先天性心脏病合并右位主动脉弓行外科手术治疗患儿的临床资料，其中男20例，女7例；年龄（10.96±12.08） 个月，体重（7.70±3.13） kg。法洛四联症14例，室间隔缺损9例，动脉导管未闭1例；肺动脉吊带1例；单纯双主动脉弓2例；均合并右位主动脉弓。其中13例合并血管环畸形，包括迷走左锁骨下动脉畸形和双主动脉弓畸形。所有患儿均接受手术治疗，在矫治心内畸形的同时，进行左锁骨下动脉移植，矫治迷走左锁骨下动脉畸形，双主动脉弓主要是切断一侧非优势弓。 结果 围术期死亡3例，其中1例术后无法停体外循环死亡，1例未合并血管环的患儿术后7 d死亡，1例合并双主动脉弓患儿术后并发急性呼吸窘迫综合征（ARDS） 死亡；1例放弃治疗。随访23例，随访时间3～17个月，心脏超声心动图提示心内畸形矫治满意，恢复良好，双侧上肢动脉波动良好。 结论 对先天性心脏病合并右位主动脉弓患者行外科矫治时应慎重，术前尽量完善相关检查，选择增强CT、磁共振成像检查，明确诊断，制定手术计划。一期同期矫治血管环畸形及其它先天性心脏病，手术效果满意，近期效果良好。

Diagnosis and Treatment of Congenital Heart Diseases with Right Aortic Arch

Objective To investigate diagnostic and treatment strategies of patients with congenital heart diseases and right aortic arch. Methods Clinical data of 27 children who underwent surgical correction for congenital heartdiseases and right aortic arch in Children’s Heart Center of Beijing Children’s Hospital from January 1，2012 to April 1，2013 were retrospectively analyzed. There were 20 male and 7 female patients with their age of 10.96±12.08 months and body weight of 7.70±3.13 kg. All the patients had right aortic arch，including 14 children with tetralogy of Fallot，9 childrenwith ventricular septal defect，1 child with patent ductus arteriosus，1 child with pulmonary artery sling and 2 children with isolated double aortic arch. Thirteen children were found to have a vascular ring including aberrant left subclavian artery and double aortic arch. All the patients received surgical correction for their intracardiac anomalies and concomitant transposition of the left subclavian artery for aberrant left subclavian artery or division of the nondominal arch for double aortic arch. Results Three children died perioperatively including 1 child who was unable to be weaned from cardiopulmonary bypass，1 child without vascular ring who died 7 days postoperatively，and 1 child with double aortic arch who died of acuterespiratory distress syndrome. There was another child who gave up further postoperative treatment. Twenty-three children were followed up for 3-17 months after discharge. Echocardiography showed satisfactory correction of their intracardiac anomalies. All the patients alive recovered well with good pulses in arteries of both arms. Conclusion Careful strategiesare needed for surgical correction of patients with congenital heart diseases and concomitant right aortic arch. Comprehensivepreoperative evaluation including contrast-enhanced CT and magnetic resonance imaging is helpful for clinical decision making in diagnosis and treatment. Clinical outcomes of 1-stage surgical correction of vascular ring and other concomitant intracardiac anomalies are satisfactory with good short-term results.