双肺移植治疗特发性肺动脉高压二例

目的 探讨双肺移植治疗特发性肺动脉高压(IPAH)的可行性及围手术期的处理方法.方法 回顾分析2例IPAH患者接受双肺移植的临床资料,2例均经右心导管术确诊为IPAH.例1为男性,17岁,体重45kg,身高165 cm,术前心功能不全Ⅳ级,肺动脉收缩压和平均压分别为110和70 mm Hg(1 mm Hg=0.133 kPa),右心房压力为18mm Hg.例2为女性,16岁,体重58 kg,身高162 cm,术前心功能不全Ⅳ级,肺动脉收缩压和平均压分别为148和72 mm Hg,右心房压力为23mm Hg.术前胸部X线片及CT显示,2例患者心脏扩大并向左偏,肺动脉增粗突起,左下肺部的血管增粗扭曲.例1和例2均在体外膜肺氧合(ECMO)支持下行序贯式双侧单肺移植术,先行右全肺切除,植入右供肺,后行左全肺切除,植入左供肺,完成双侧前外侧切口不横断胸骨序贯式双肺移植术.供、受者体型较为匹配,ABO血型相同或相容,供肺冷缺血时间为185～300 min.例1和例2术中ECMO支持时间分别为550和450 min,出血量分别为3000和1200 ml.结果 术后12 h患者胸部X线片和CT显示双侧移植肺清晰,心脏大小恢复正常.例1和例2分别在术后16和13 h撤离ECMO;术后第3和第4天均出现不同程度的血液动力学不稳定,发生急性左心功能衰竭,于术后第3和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和12天脱离呼吸机,术后第93和32天康复出院.出院时,心脏超声检查显示,患者心脏形态及心功能明显改善,心功能达Ⅰ级.2例患者分别随访25和10个月,已恢复正常的工作和学习,生活质量良好.结论 对于终末期IPAH经内科保守治疗效果欠佳的患者行序贯式双肺移植是可行的.良好的供肺切取和保护技术、术中ECMO的支持以及术后左心功能衰竭的防治是手术成功的关键.

Bilateral lung transplantation for idiopathic pulmonary artery hypertension

Objective To evaluate the operative technique, perioperative management and outcomes of bilateral lung transplantation for idiopathic pulmonary artery hypertension. Methods There were 2 cases of idiopathic pulmonary hypertension subject to bilateral sequential lung transplantation with ECMO support (16, 17 years old, respectively). The pulmonary artery pressure was 10/70 and 148/72 mm Hg respectively. The heart function was NYHA Ⅳ, the operative procedure was right lung first, then left lung, and the cold ischemia time was 230 min/430 min and 185 min/300 min respectively. The ECMO support time was 550 min and 450 min respectively. The blood loss during the operation was 3000 and 1200 ml, respectively. Resuits The ECMO was withdrawn 16 and 13 h postoperation respectively. There was unstable hemadynamics and acute left heart failure on the 3rd and 4th day after the operation. The patients were treated with ventilate support and tracheotomy on 3rd and 6th day respectively. Additionally, the patients were given cardiotonic, dieresis and the ventilation was withdrawn on 33rd and 12th day after the operation respectively. They were discharged from the hospital on 93rd and 32nd day after the operation. The heart function both reached NYHA I, two cases were followed up for 25 and 10 months respectively.Both of them had an excellent quality life. Conclusion Bilateral lung transplantation is effective for end-stage idiopathic pulmonary artery hypertension even with slight right ventricular dysfunction with satisfactory short-term outcome. A limited operative time, an ECMO support for heart and lung during the peri-operative period and a perfect management for the left ventricular dysfunction after surgery are key roles for the success. Closed follow-up and surveillance are needed for long-term outcomes.