完全性肺静脉异位引流伴梗阻的治疗效果探讨

Treatment of complete anomalous pulmonary venous drainage with obstruction

目的评估完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)伴梗阻的治疗效果。方法回顾性分析2010年1月至2019年12月浙江大学医学院附属儿童医院收治的189例TAPVC且接受手术治疗的患儿临床资料,其中TAPVC合并梗阻(梗阻组)36例,TAPVC未合并梗阻(非梗阻组)153例;梗阻组中,男20例,女16例;年龄1~140(29.1±28.7)d;手术时体质量2.4~6.5(3.6±0.9)kg。根据术后是否死亡,将梗阻组36例进一步分为梗阻死亡组(5例)和梗阻生存组(31例);比较两组术前血液乳酸水平、术前左室舒张末期容积指数、体外循环时间、术后机械通气时间,以及术后体外膜肺氧合(extracorporeal membrane oxygenation,ECMO)支持、延迟关胸、多功能脏器衰竭、腹膜透析、心律失常、术后肺高压危象以及肺静脉梗阻的人数比例。结果梗阻组与非梗阻组年龄分别为(29.1±28.7)d和(98.3±121.6)d;体重分别为(3.6±0.9)kg和(5.1±3.4)kg;新生儿占比分别为58.3%(21/36)和20.9%(32/153);术后肺静脉入左房流速分别为(1.4±0.5)m/s和(1.1±0.4)m/s;延迟关胸例数分别为15例(15/36,41.6%)和10例(10/153,6.5%);死亡例数分别为5例(5/36,13.9%)和10例(10/153,6.5%);两组年龄、体重、新生儿占比、术后肺静脉入左房流速、延迟关胸占比和死亡率差异均有统计学意义(P<0.05)。梗阻死亡组与梗阻生存组的术前血液乳酸分别为(6.7±3.8)mmol/L和(2.5±1.2)mmol/L;左室舒张末期容积指数分别为(27.1±19.5)mL/m^(2)和(29.2±16.8)mL/m^(2);体外循环时间分别为(216.3±64.3)min和(159.3±73.4)min;术后机械通气时间分别为(17.5±13.5)d和(4.8±5.2)d;行ECMO支持例数分别为3例和1例;延迟关胸例数分别为4例和11例;多脏器功能衰竭例数分别为3例和0例;腹膜透析例数分别为4例和16例;心律失常例数分别为2例和3例;术后肺高压危象例数分别为3例和1例;肺静脉梗阻例数分别为2例和4例;两组术前血液乳酸水平、体外循环时间、术后机械通气时间、ECMO支持、多功能脏器衰竭及术后肺高压危象差异均有统计学意义(P<0.05)。生存患儿随访6个月无一例出现肺静脉梗阻。结论完全性肺静脉异位引流伴梗阻患儿手术死亡率高,术中可应用各种技术扩大吻合口,早期死亡与多脏器功能衰竭有关,围手术期应用ECMO、延迟关胸及腹膜透析能降低病死率,中远期死亡与肺静脉梗阻有关。

Objective To summarize and evaluate the therapeutic effect of total anomalous pulmonary venous drainage with obstruction.Methods The clinical data of 189 children treated with TAPVC surgery in Children's Hospital Affiliated to Zhejiang University School of Medicine from January 2010 to December 2019 were retrospectively analyzed,including 36 cases in the obstruction group and 153 cases in the non-obstruction group.The obstruction group contained 20 males and 16 females with age 1~140(29.1±28.7)d and body weight at the time of operation 2.4~6.5(3.6±0.9)kg.According to the postoperative death,the patients were further divided into the obstruction death group(5 cases)and the obstruction survival group(31 cases).Compare two groups of preoperative lactic acid levels,preoperative left ventricular end-diastolic volume index,extracorporeal circulation time,postoperative mechanical ventilation time,postoperative Extracorporeal Membrane Oxygenation(ECMO)support cases,delayed chest closure cases,multifunctional organ failure cases,peritoneal dialysis cases,arrhythmia cases,postoperative pulmonary hypertension crisis cases and pulmonary venous obstruction cases.35 cases were done with the vertical veins ligation and establishment of the connection between pulmonary vein and left atrium,without sutureless technique of pericardium in situ.Results Comparing the obstruction group and the non-obstruction group,ages were(29.1±28.7)vs.(98.3±121.6)days,body weight(3.6±0.9)vs.(5.1±3.4)kg,neonatal cases 21(58.3%)vs.32(20.9%),pulmonary venous velocity(1.4±0.5)vs.(1.1±0.4)m/s,delayed chest closure cases 15(41.6%)vs.10(6.5%),death cases 5(13.9%)vs.10(6.5%).There were statistically significant differences in age,body weight,neonatal proportion,pulmonary venous velocity,delayed chest closure and death between the two groups(P<0.05).Comparing the obstruction death group and the obstruction survival group,lactic acid levels were(6.7±3.8)vs.(2.5±1.2)mmol/L,left ventricular end-diastolic volume indices(27.1±19.5)vs.(29.2±16.8)mL/m^(2),extracorporeal circulation time(216.3±64.3)vs.(159.3±73.4)mins,postoperative mechanical ventilation time(17.5±13.5)vs.(4.8±5.2)d,ECMO support cases 3(60%)vs.1(3.2%),delayed chest closure cases 4(80%)vs.11(35.5%),multifunctional organ failure cases 3(60%)vs.0,peritoneal dialysis cases 4(80%)vs.16(51.6%),arrhythmia 2(40%)vs.3(9.7%),pulmonary hypertension crisis cases 3(60%)vs.1(3.2%),pulmonary venous obstruction cases 2(40%)vs.4(12.9%)respectively.There were statistically significant differences in preoperative lactic acid,extracorporeal circulation time,postoperative mechanical ventilation time,ECMO support cases,multifunctional organ failure cases and postoperative pulmonary hypertension crisis cases between the two groups(P<0.05).No pulmonary vein obstruction was found in the surviving children after 6 months of follow-up.Conclusion The operative mortality of children with complete pulmonary vein obstruction was high.Various techniques were used to expand the anastomosis during the operation.The early death was related to multiple organ failure and the perioperative application of ECMO,delayed chest closure and peritoneal dialysis can reduce the mortality.Pulmonary vein obstruction induced to the medium and long-term death.