肺动脉瓣缺如综合征外科治疗

Surgical management of absent pulmonary valve syndrome

目的回顾性研究肺动脉瓣缺如综合征围手术期处理及手术操作技术，探讨减轻术后呼吸系统并发症的综合措施。方法2005年1月至2012年1月，11例肺动脉瓣缺如综合征患儿接受体外循环下畸形矫治手术，其中男7例，女4例；年龄1～5岁；体质量10．2～17．5kg，平均（12．3±3．4）kg。手术选择右心室流出道切口，应用经戊二醛处理的自体心包补片修补室间隔缺损；修剪主肺动脉及左、右肺动脉前壁冗余组织至双侧肺门，缝合重塑肺动脉至正常内径；应用0．1mmGore—Tex心包膜在右心室流出道切口处制备人工肺动脉单瓣后，应用自体心包行跨瓣环补片，扩大右心室流出道及肺动脉瓣环。对于支气管压迫严重的患儿，采用Lecompte技术将肺动脉移至升主动脉前方。术中及术后早期行支气管纤维镜检查，判断气管压迫的位置、程度以及是否存在气管发育不良，并充分灌洗气道，将其中积聚的分泌物吸出。5例术中及术后应用纤维支气管镜探查支气管并清理气道分泌物，5例采用Lecom—pte技术以减轻肺动脉对于左、右支气管的压迫，4例应用深低温停循环技术。术后随访3～87个月，中位数为39个月，分别于出院前、术后3个月和之后每年1次常规心脏超声随访检查。结果全组无死亡。随访未见心脏残余畸形。2例患儿术后1年内反复出现肺部感染，之后改善；行Lecompte调转的患儿支气管无肺动脉压迫性狭窄。所有患儿无气道压迫症状。结论对于肺动脉瓣缺如综合征，理想的肺动脉重建、减轻肺动脉对同侧支气管的压迫及应用纤维支气管镜辅助检查及治疗是获得理想疗效的基本保障措施。但是由于分支肺动脉的瘤样病变可能会对伴行细支气管造成压迫，因此患儿术后仍可能面临较长时间的内科治疗，其远期疗效尚待进一步研究。

Objective The aim of this study is to retrospectively analyze surgical management of absent pulmonary valve syndrome（APVS）. Methods Between January 2005 and January 2012, totally 11 children with APVS underwent primarysurgical correction. There were 7 boys and 4 girls. Age at surgeries range from 1 to 5 years, and weight from 10.2 -17.5 kg, with average （12.3 ± 3.4） kg. Surgical procedures include VSD repair, pulmonary arteries reconstruction and RVOT reconstruction with monocusp valve. 5 cases chose Lecompte maneuver as an option to release compression to bronchus, 5 cases underwent fibroscopy inspection and airway secretion suction, and 4 cases adapted deep hypothermic circulatory arrest （DHCA）during correction procedures. All survivors are routinely followed-up with echocardiogram. Results All 11 cases survived, 2 of them suffered from frequent lungs infections during the first year post surgery. No case exists bronchus or pulmonary arteriescompression. Conclusion APVS is a rare congenital heart defect, which may challenge perioperative managements and operations. Ideal surgical correction includes RVOT reconstruction, decompression of bilateral bronchus, and airway inspection withfibroseopy. However, compression of intrapulmonary bronchi by abnormally branching pulmonary arteries may expose patients to a relative long time of medication therapy after surgical correction. A large number of patients with long-term follow-up are needed to draw definitive conclusions on this strategy＇ s effectiveness.