肺移植治疗特发性肺间质纤维化32例报告

目的 探讨肺移植治疗特发性肺间质纤维化(IPF)的手术适应证、手术方式及疗效.方法 76例肺移植中32例为肺移植治疗IPF.移植前,患者均经高分辨螺旋CT检查显示为典型的弥漫性蜂窝肺,经开胸或纤维支气管镜肺活组织病理检查确诊为IPF的有16例,动脉血气分析显示动脉血氧分压(PaO_2)≤50 mm Hg(1 mm Hg=0.133 kPa)的有28例,平均肺动脉压≥30 mm Hg的有15例.32例IPF患者中,28例接受单肺移植,其中常规体外循环下完成2例,体外膜肺氧合(ECMO)支持下完成14例;4例双肺移植均在ECMO支持下进行,取双侧前胸切口不横断胸骨,依次完成双侧单肺移植.术后对所有受者进行了随访,观察患者的预后情况.结果 所有受者均顺利完成手术.18例应用ECMO辅助的受者中,术后有17例成功撤除ECMO辅助,1例于术后2周因多器官功能衰竭而死亡.除上述死亡的1例外,术后早期还有4例死亡,1例术后发生支气管吻合口漏,经手术修补后发生吻合口裂开而死亡,1例死于移植肺活动性出血,2例死于移植肺功能丧失.术后1年内有12例发生急性排斥反应,发生率为37.5%,1例因重症排斥反应死亡,2例因严重感染死亡.受者术后3个月、1年及3年存活率分别为84.3%、75%和54.5%,中位存活时间为51个月,受者肺功能改善明显,生活质量显著提高.结论 肺移植是治疗IPF的有效方法,术后可明显延长受者存活时间,单肺移植可作为IPF的常用术式.对年龄较轻者应选择双肺移植.

The technical experience of lung transplantation for idiopathic pulmonary fibrosis (32 eases report)

Objective To estimate the operative technique, operative indications and the outcome of lung transplantation (LT) for idiopathic pulmonary fibrosis (IPF).Methods Of 76 cases receiving LT in our hospital, there were 32 cases of IPF, include 6 females and 26 males with age ranging from 22 to 74 (mean 57.5±12.3 years old).The high resolution CT showed diffuse honeycomb lung.Sixteen cases were diagnosed histopathologically.All of them were oxygen-dependent.The mean pulmonary artery pressure (mPAP) was ≥30 mm Hg (15 cases), PaO_2≤50 mm Hg (28 cases).The surgical procedures included single LT (28 cases) and double LT (4 cases) which were performed via two sequential anterolateral thoracotomies.Twenty cases of them received the ECMO (18/20) support or CPB support (2/20).Results The surgical procedures were done successfully.17 cases postoperative were successfully removed The ECMO was successfully removed postoperatively in 17 cases, and the rest one was died at the 2nd week after the operation due to MODS.There were 4 deaths in the early period (0-30 days ): one due to active bleeding in transplanted lung, 2 due to graft failure, and 1 due to anastomotic bronchial leak at the 2nd week after the operation, and anastomotic bronchial dehiscence at the 8th day after the reoperation.There were 12 cases of acute rejection within postoperative one year.One of them was died of severe acute rejection (A4).Two cases were died of infection.Three-month, one-year, and 3-year overall survival rate for recipients of lung transplants was 84.3 %, 75 %, and 54.5 % respectively.The overall survival half-life was 51 months.Conclusion LT is effective in improving the survival of selected patients with IPF.It can prolong the life expectancy and improve the living quality.Single lung transplant was a routine way for IPF patient.We should select bilateral lung transplant for younger and infected patients.