CAJ | 학술논문

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.
목적 탐토쌍폐이식치료특발성폐동맥고압(IPAH)적가행성급위수술기적처리방법.방법 회고분석2례IPAH환자접수쌍폐이식적림상자료,2례균경우심도관술학진위IPAH.례1위남성,17세,체중45kg,신고165 cm,술전심공능불전Ⅳ급,폐동맥수축압화평균압분별위110화70 mm Hg(1 mm Hg=0.133 kPa),우심방압력위18mm Hg.례2위녀성,16세,체중58 kg,신고162 cm,술전심공능불전Ⅳ급,폐동맥수축압화평균압분별위148화72 mm Hg,우심방압력위23mm Hg.술전흉부X선편급CT현시,2례환자심장확대병향좌편,폐동맥증조돌기,좌하폐부적혈관증조뉴곡.례1화례2균재체외막폐양합(ECMO)지지하행서관식쌍측단폐이식술,선행우전폐절제,식입우공폐,후행좌전폐절제,식입좌공폐,완성쌍측전외측절구불횡단흉골서관식쌍폐이식술.공、수자체형교위필배,ABO혈형상동혹상용,공폐랭결혈시간위185～300 min.례1화례2술중ECMO지지시간분별위550화450 min,출혈량분별위3000화1200 ml.결과 술후12 h환자흉부X선편화CT현시쌍측이식폐청석,심장대소회복정상.례1화례2분별재술후16화13 h철리ECMO;술후제3화제4천균출현불동정도적혈액동역학불은정,발생급성좌심공능쇠갈,우술후제3화6천행기관절개호흡궤보조정압통기,경강심、이뇨、확혈관등치료,분별우술후제33화12천탈리호흡궤,술후제93화32천강복출원.출원시,심장초성검사현시,환자심장형태급심공능명현개선,심공능체Ⅰ급.2례환자분별수방25화10개월,이회복정상적공작화학습,생활질량량호.결론 대우종말기IPAH경내과보수치료효과흠가적환자행서관식쌍폐이식시가행적.량호적공폐절취화보호기술、술중ECMO적지지이급술후좌심공능쇠갈적방치시수술성공적관건.
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 Resuits The ECMO was withdrawn 16 and 13 h postoperation respectively.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.