小儿先天性三房心外科诊治经验

Experience of surgical repair for cor triatriatum sinister in chirdren

目的总结近年来先天性三房心的诊治经验。方法2008年7月至2013年6月24例先天性三房心纠治手术患儿，均手术中探查证实诊断，接受全麻体外循环下三房心纠治手术，术时最小年龄1个月，中位数5个月；最小体质量3．5kg，中位数7．4kg。回顾性研究病例资料，手术生存患儿定期门诊随访，观察恢复情况及手术效果。结果24例患儿中诊断单纯三房心4例，合并其他畸形20例。22例手术一期纠正所有合并心脏畸形，1例行三房心纠治合并Glenn手术，1例功能性单心室Glenn术后仅行三房心纠治手术。2例术后早期出现肺动脉高压危象。1例多器官畸形患儿术后17天因呼吸衰竭死亡。23例患儿恢复良好出院，心功能Ⅰ～Ⅱ级，门诊随访最长73个月。术后12个月复查超声心动图，患儿左心房直径与同年龄段正常儿童平均值差异无统计学意义，P=0．86。截止2015年4月，无患儿再次干预，未发现肺静脉、左房及二尖瓣水平的血流梗阻。结论确诊三房心的患儿，无论是否存在血流动力学意义，均建议考虑手术治疗，正确选择手术时机尤为重要。及时诊断、积极治疗三房心效果良好。

Objective To share the experience of surgical repair for cor triatriatum in chidren. Methods Between July 2008 and June 2013,24 children with cor triatriatum underwent surgical correction in Cardiac Center of Children＇ s Hospital of Fudan University. The minimum age at the time of operation was 1 month, median age was 5 month ; the minimum body weight at the time of operation was 3.5 kg, median body weight was 7.4 kg. Retrospectively analyzed their cardiac anatomy, clinical data, surgical procedures and follow-up data. Results Of all 24 patients, there were 4 patients diagnosed sempliee cor triatria- turn, and 20 patients diagnosed cor triatriatum associated with other malformation includes cardiac and non-cardiac issues. Pro- cedures were performed on 22 patients to correct all cardiac defects in one stage. 1 patient underwent surgical repair for cor tria- triatum and Glenn procedure as well. 1 patient, diagnosed as cor triatriatum and functional single ventricle S/P Glenn, under- went surgieal repair for eor tiratriatum only. 2 patients recovered from pulmonary artery hypertension crisis after operation. 1 pa- tient dead 17 days pest procedure caused by respiratory failure. 23 patients discharged from hospital. The longest follow-up was 73 months, at least 12 months after discharge. All alive patients were in New York Heart Association＇ s function class I-II. Echo assessment revealed normal size of left atriums of almost all alive patients one year after discharge. Till April 2015, no postoperation intervention, no blood flow obstruction at level of pulmonary veins, left atrium or mitral valve. Condusion Ped- iatric patients diagnosed cor tiratriatum sinister need intervention, no matter hemodynamie ehange occurs or not. Right time of surgery is important for successful treatment.