肺移植治疗儿童肺动脉高压的临床疗效

目的 探讨同种异体肺移植治疗儿童肺动脉高压(PAH)的临床疗效.方法 受者例1、2为特发性肺动脉高压( IPAH)患儿,均经右心导管术确诊为IPAH,术前心功能不全Ⅳ级,肺动脉收缩压、平均压分别为110、70 mm Hg(1 mm Hg =0.133 kPa)和148、72 mm Hg,在全麻体外膜肺氧合(ECMO)支持下行序贯式双侧单肺移植术,术中ECMO支持时间分别为550 min和450 min,出血量分别为3000 ml和1200 ml.受者例3为先天性心脏病室间隔缺损合并艾森曼格综合征心内直视探查术后,超声心动图(UCG)提示先天性心脏病室间隔缺损,双向分流,肺动脉收缩压、平均压为110、60 mm Hg,在全麻低温体外循环(CPB)下行室间隔缺损修补术同期右侧单肺移植术,术中CPB时间244 min.3例受者与供者体型较匹配,ABO血型相同.结果 受者例1、2术后ECMO分别维持16h、13 h后撤离,术后第3、4天均出现不同程度的血流动力学不稳定,诊断为急性左心衰,均于术后第3、6天行气管切开呼吸机辅助正压通气,经强心、利尿、扩血管等治疗,分别于术后第33天、12天脱离呼吸机.受者例3术后3天内移植肺出现中等程度再植反应性肺水肿,术后第7天气管切开,第12天撤离呼吸机；术后第14天出现急性排斥1次,治疗后缓解.3例受者术后UCG提示心脏形态和心功能明显改善,受者例3室间隔缺损修补完整,无残余分流.分别于术后第93天、32天、62天康复出院,心功能均达Ⅰ级,肺动脉收缩压、平均压分别降为54、32 mm Hg,60、36 mm Hg和53、39 mm Hg.术后已随访41、21、82个月,患儿正常工作学习,至今生活质量良好.结论 对终末期经内科保守治疗效果欠佳的PAH患儿行肺移植能很好改善生活质量.

The clinical effect of lung transplantation for pediatric pulmonary artery hypertension

Objective To evaluate the clinical effect of lung transplantation for pediatric pulmonary artery hypertension (PAH).Methods The recepter 1 and 2 with idiopathic pulmonary artery hypertension (IPAH) were diagnosed by right catheterization.The heart fuction was NYHA IV.The pulmonary artery pressure and mean pulmonary artery pressure were 110/70mmHg and 148/72 mmHg respectively.They underwent bilateral sequential lung transplantation with extracorporeal membrane oxygenation (ECMO) support.During operation,the ECMO support time was 550 min and 450 min and the blood loss was 3000 ml and 1200 ml respectively.The recepter 3 with end-stage congenital ventricular septal defect with Eisenmenger syndrome (ES) who had received open heart exploration underwent right single lung transplantation and ventricular septal defect repair under cardiopulmoanry bypass(CPB).There were ventricular septal defect and bidirectional shunt through UCG assessment The pulmonary artery pressure and mean pulmonary artery pressure were 110/60 mmHg.CPB time was 244 min.The three recepters had the same ABO blood group and the similar body type with the three donors.Results The recepter 1 and 2 was sustained by ECMO after operation for 16 h and 13h respectively.But unstable hemadynamics and acute left heart failure occured on 3rd and 4th day after the operation respectively.We treated them with ventilate support and tracheotomy on 3rd and 6th day respectively.They were also treated with cardiotonic; dieresis and the patients were weaned away from the ventilation on 33rd and 12ed day after the operation respectively.The transplanted lung of the receptor 3 had pneumochysis in the first 3 days.The patient was treated with ventilate support and tracheotomy on 7th day and was weaned from the ventilation on 12ed day after the operation.An acute rejection episode occurred on 14th day.The cordioform and heart function of the three cases improved and especially the receptor 3 had intact repaired of ventricular septal defect.They were discharged from the hospital on 93rd,32ed and 62ed day afer the operation.The heart function all reached NYHA I and the pulmonary artery pressure and mean pulmonary artery pressure was reduced to 54/32 mmHg,60/36 mmHg and 53/39 mmHg respectively.The three cases have been followed up for 41 months,21 months,and 82 months.They are having an excellent quality life.Conclusion Lung transplantation is effective to improve the quality of life for end-stage pediatric pulmonary artery hypertension even with slight right ventricular dysfunction with satisfying short-term results.