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目的 探讨左心室大小对婴儿完全性肺静脉异位引流(TAPVC)解剖矫治手术早期结果的影响.方法 2010年1月至2013年6月,103例1岁以内TAPVC患婴行双心室矫治,男65例,女38例;体质量(5.3 ±1.3) kg.以左心室舒张末期容积指数(LVEDVI) 20 ml/m2为标准,将全部患婴分为小左心室组和接近正常左心室组.比较两组患婴的年龄、体质量、病理分型、合并肺静脉梗阻及限制性房间隔缺损;以Z值对照两组患婴较正常婴儿左心房、左心室的减小程度.在胸部正中切口,中度低温体外循环下行TAPVC矫治术,同期矫治合并畸形.结果 小左心室组45例,接近正常左心室组58例.小左心室组合并梗阻比例达71.1％,显著高于接近正常左心室组.小左心室组左心室舒张末径Z值显著低于接近正常左心室组患婴.两组体外循环(96.6±34.4) min,主动脉阻断(58.0±21.1) min.术后早期死亡4例,占3.9％,无因小左心室导致低心排血量综合征死亡患婴.小左心室组术后呼吸机辅助时间、ICU停留时间及血管活性药物应用时间均显著长于接近正常左心室组.结论 小左心室梗阻型TAPVC患婴只要其二尖瓣、主动脉瓣发育无明显减小,则无需考虑左心室减少程度而均可施行解剖矫治,术中、术后早期预防及处理低心排血量综合征是取得良好手术结果的关键.

Objective To investigate the impacts of the left ventricular size for infants with total anomalous pulmonary venous connection(TAPVC) on the early results of anatomical correction.Methods From Jan 2010 to Jun 2013,103 cases of TAPVC children under 1 year of age received biventricular correction in our hospital,including 65 males and 38 females with the mean body weight of(5.3 ± 1.3) kg.Taking left ventricular end-diastolic volume index(LVEDVI) of 20 ml/m2 as a boundary,all the children were divided into two groups:"Small LV "group and the "Near normal LV " group.Various factors including age,body weight,pathological type,pulmonary venous obstruction and restricted atrial septal defect were compared between the two groups.The "Z value" were introduced to demonstrate the small extent of the left atrium and left ventricle of TAPVC patients in comparison with the normal children.TAPVC correction surgery were performed with conventional median sternotomy,moderate hypothermic cardiopulmonary bypass and combined malformations were treated simultaneously.Results 45 patients were classified to "Small LV" group and 58 patients were classified to " Near normal LV" group.71.1％ of all "Small LV" patients was diagnosed as the obstruction type of TAPVC,the ratio was significantly higher than that of the " Near normal LV" group.The "Z value" of left ventricular end-diastolic diameter in the " Small LV" group was significantly lower than that of the "Near normal LV" group.The mean CPB and aortic clamping time of all patients were (96.6 ± 34.4) min and (58.0 ±21.1) min respectively.There were 4 early postoperative death and the overall mortality was 3.9％.No patient was dead of low cardiac output.The duration of postoperative mechanical ventilation,ICU stay and vasoactive drugs application in "Small LV" group was significantly longer than that of "Near normal LV" group.Conclusion The " Small LV",which should be viewed as "relative dysplasia of left ventricle ",is more common in obstructive type of TAPVC.As long as the sizes of mitral valve and aortic valve were not significantly reduced,anatomic correction can be implemented and need not to concern the reducing degree of left ventricle.Nevertheless,the prevention and treatment of low cardiac output in the operation and early postoperative period were still key points for "small LV" patients to achieve good surgical results.