单中心328例不同分型完全性肺静脉异位引流的临床特点和外科治疗早中期结果  

Early-and intermediate-term results of surgical correction in 328 patients with different drainage type of total anoma-lous pulmonary venous connection

作　　者：欧艳秋[1] 聂志强[1] 庄建[2] 陈寄梅[2] 高向民[1] 吴勇[1] 曲艳吉[1] 麦劲壮[1] 李晓华[2] 刘小清[1] 岑坚正[2] 

机构地区：[1]广东省心血管病研究所广东省华南结构性心脏病重点实验室,广州510080 [2]广东省医学科学院心脏外科

出　　处：《中华胸心血管外科杂志》2017年第1期10-15,共6页Chinese Journal of Thoracic and Cardiovascular Surgery

基　　金：国家“十二五”科技支撑计划（2011BAI11B22,2012BA104805）,国家自然科学基金（U1401255）,广东省科技计划国际合作项目（2014A050503048）

摘　　要：目的：分析单中心完全性肺静脉异位引流（ TAPVC）外科治疗不同分型的早、中期效果。方法连续入选2006年1月至2013年12月328例行外科矫治术的TAPVC患者，其中心内型109例，心上型161例，心下型32例和混合型26例。收集术前、术后临床资料并随访至2015年5月。数据统计应用SPSS 21.0软件。不同分型组间比较采用χ2检验。 Cox单因素和多因素回归分析不同类型外科治疗TAPVC与术后死亡和肺静脉梗阻（ PVO）关系。生存及术后无PVO情况采用Kaplan-Meier生存分析，组间比较采用log-rank检验。结果不同分型的基线资料差异明显，心下型患者的基线情况最为危重，包含新生儿、术前Ⅲ～Ⅳ级心功能、重度肺高压和三尖瓣反流、术前PVO的比例最高，体质量和年龄最小。心下型和混合型的体外循环、主动脉阻断和术后机械通气时间明显多于心内型和心上型，差异有统计学意义。心下型（9.4％）和混合型（11.5％）手术死亡比例比心内型（4.6％）和心上型（7.5％）高，但差异无统计学意义，中期死亡比例心下型21.9％、混合型30.8％，明显高于心内型（8.3％）和心上型（11.8％）；术后需要再干预的PVO发生率混合型（19.2％）最高，其次是心下型（15.6％）。经过校正混杂因素后，心下型和混合型相对于其他两种类型是TAPVC患者术后死亡和发生PVO的独立危险因素。结论 TAPVC不同分型的早期和中期预后有明显差异，心下型和混合型是术后死亡和PVO发生的独立危险因素。Objective This retrospective cohort study aims to evaluate and compare the prognosis of surgical repair for total anomalous pulmonary venous connection（TAPVC） with different drainage type.Methods From January 2006 to Decem-ber 2013, 328 consecutive patients were enrolled in this study .The distribution of the defects was 109 cases with cardiac, 161 with supracardiac, 32 with infracardiac, and 26 with mixed type of the drainage into the systemic circulation .The clinical re-cords of all the patients were reviewed.Studied variables were extracted from the clinical records.Followed-up was conducted at an interval of 1 month, 3 months, 6 months and then once a year post-operation.Prevalence of peri-operative conditions were compared among four different types.Studied endpoints was defined by postoperative total death or pulmonary venous obstruc-tion（PVO）, which was evaluated with Kaplan-Meier curve and multivariable Cox proportional hazard model, adjusted by differ-ent surgical strategy, emergency operation, preoperative-PVO, neonates, weight, combing with other complex cardiac defects,NYHA cardiac function, severe pulmonary hypertension and severe tricuspid regurgitation.Results There were significant discrepancyof preoperative conditions among four types of TAPVC.Patients with infracardiac TAPVC presented the most criticalsymptoms and clinical indexes, which included having largest proportion of neonates, preoperative PVO, severe NYHA grading,pulmonary hypertension and tricuspid regurgitation, having lowest body weight at operation and youngest age.The cardiopulmonarybypass time, aortic crossclamp time and mechanical ventilation time were significantly longer in infracadiac and mixedTAPVC comparing to the other two types.For early mortality（death in hospital）, infracadiac（9.4%） and mixed（11.5%）TAPVC demonstrated higher rates of death than cardiac（4.6%） and supracardiac（7.5%）TAPVC, although had no statisticalsignificance.For intermediate-term results, mortality in infracadiac（21.9%） a

关 键 词：心脏外科手术 全肺静脉异位引流 肺静脉梗阻 

分 类 号：R726.5[医药卫生—儿科]