胸腔不匹配受者肺叶移植治疗终末期肺病34例

Clinical analysis of bilateral lobar lung transplantation on end-stage lung disease in patients with size mismatching thoracic cavity

目的探讨胸腔不匹配受者行肺叶移植治疗终末期肺病的有效性和安全性，分析其预后。方法回顾性分析34例肺叶移植受者相关资料，其中男性14例，女性20例，年龄为（37．0±6．3）岁（15-56岁）。患者原发病为特发性肺间质纤维化24例，先天性房室间隔缺损并发艾森门格综合征3例，特发性肺动脉高压2例，肺淋巴管平滑肌瘤病3例，支气管扩张2例。26例在体外膜肺氧合（ECMO）支持下完成移植（静脉-动脉模式），术前行ECMO辅助3例。右肺中下叶联合左下肺叶移植24例，双侧下叶移植4例，左肺劈离式双侧肺叶移植4例，双侧上叶移植2例。双侧移植肺冷缺血时间分别为（4．4±1．2）h和（6．3±1．4）h。结果术后早期（30d内）死亡8例，占23．5％。死亡原因为移植物功能丧失2例，多器官功能衰竭2例，绿脓假单胞菌感染1例，巨细胞病毒感染1例，脓毒血症2例。术后因原发性移植物功能丧失、严重低氧血症、急性肺水肿行ECMO辅助循环10例。脑出血1例；静脉吻合口狭窄梗阻2例，均再次开胸手术；心功能衰竭3例；肾功能衰竭2例；支气管胸膜瘘1例；腹股沟处淋巴管瘘3例；右下肢动脉血栓1例。术后3～6个月内复查23例受者的肺功能，第一秒用力呼气量、最大通气量分别占预期值的（74．23±4．86）％和（72．0±3．64）％。术后1、2、3、5年累积存活率分别为70．9％、60．8％、50．6％和36．2oA。结论对于胸腔不匹配患者，肺叶移植治疗终末期肺病安全有效，能够显著改善肺功能和预后。

Objective To investigate the effectiveness and safety of bilateral lobar lung transplantations （LLTx） in thoracic cavity size mismatching patients with end-stage lung diseases and analyze its prognosis. Method Clinical data of 34 patients receiving LLTx in our hospital were retrospectively analyzed between Sept. 2002 to Dec. 2012. LLTx were performed on 14 male and 20 female patients, with a mean age of 37. 0±6. 3 years （ranging from 15 to 56 years）. Indications for LLTx were idiopathic pulmonary fibrosis （n = 24）, idiopathic pulmonary hypertension （n = 2）, Eisenmenger syndrome （n = 3）, bronchiectasis （n = 2） and lymphangiomyomatosis （n = 3）. Amongst them 26 patients received LLTx under extracorporeal membrane oxygenation （ECMO） support and 3 on ECMO as a bridge to transplantation. Twenty-four middle/lower right lobes with left lower LLT, 4 bilateral lower LLT, 2 bilateral superior LLT and 4 split left lung LLT were performed. Ischemie time was 4. 4 ± 1.2 h for the first lobe and 6. 3±1.4 h for the second. Result The mortality in postoperative prophase （30 days） was 23.5%. The 1-, 2-, 3- and 5-year survival rate after LLTx was 70.9%, 60.8%, 50. 6% and 36. 2% respectively. The main causes of mortality included primary graft dysfunction, acute rejection, multiple organ failure, bronchiolitis obliterans and sepsis. Lung function test was performed on 23 cases after transplantation and forced expiratory volume in first second, maximal voluntary ventilation accounting for the predicted value was （74. 23 ± 4. 86） % and （72. 0 ± 3. 64）%, respectively. Conclusion This study demonstrates that for thoracic cavity size mismatching patients, LLTx are safe and effective in the treatment of end-stage lung diseases, which can ameliorate the lung function and prognosis.