Bech Bo, Pressler Tanja, Iversen Martin, Carlsen Jørn, Milman Nils, Eliasen Kirsten, Perko Mario, Arendrup Henrik
Department of Cardiothoracic Surgery, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
Eur J Cardiothorac Surg. 2004 Dec;26(6):1180-6. doi: 10.1016/j.ejcts.2004.08.015.
Over the last decades improvements in medical therapies have delayed the progression of lung disease in cystic fibrosis (CF). However, lung disease is still the most common cause of premature death, and lung transplantation today is the only treatment for end-stage lung disease in patients with CF. We present a retrospective review of the outcome of CF patients transplanted in Denmark since start of the national lung transplantation programme in 1992.
In a 10-year period, 47 patients with CF were listed for lung transplantation; 29 patients underwent transplantation and 18 patients died while waiting for donor organs. Eleven patients received en block double lung transplantation with direct bronchial artery revascularization and 18 patients received bilateral sequential lung transplantation. Median age at transplantation was 29 years (range 11-50).
The perioperative mortality (< or =30 days) was 3.5% (1/29 patients). Actuarial survival of transplanted patients at 1, 3, 5 and 8 years was 89, 80, 80 and 70%, respectively. Actuarial survival of non-transplanted patients on the waiting list at 1 and 2 years was 28 and 11% (P<0.0001). Causes of death of transplanted patients were: respiratory failure on day 7 (n=1), bronchiolitis obliterans syndrome (n=2), infection (Cytomegalovirus, Aspergillus fumigatus) (n=2), bronchial anastomosis dehiscence (n=1). Pulmonary function (FEV1% predicted) improved from median 20% (range 13-31) pre-transplant to 71% (range 19-118) after 5 years (P<0.0001). Renal function (51Cr-EDTA clearance) decreased from median 97 ml/min (range 45-190) pre-transplant to 32 ml/min (range 8-84) 6 months after transplantation (P<0.001). Three patients (11%) received dialysis post-transplant of whom two underwent kidney transplantation. Immunosuppressive induction therapy with rabbit-antithymocyte-globulin compared to daclizumab resulted in fewer treatments for acute rejection within the first 3 months post-transplant (P=0.05 at 5-8 weeks). Burkholderia multivorans was present in three patients pre-transplant with satisfying long-term outcome in one patient.
Lung transplantation is a well-established life-extending treatment for patients with CF and end-stage lung disease. The operative mortality is low and CF patients have a significant early survival benefit after lung transplantation. Satisfying long-term results can be achieved in this young and severely ill group of patients.
在过去几十年中，医学治疗方法的改进延缓了囊性纤维化（CF）患者肺部疾病的进展。然而，肺部疾病仍然是过早死亡的最常见原因，如今肺移植是CF患者终末期肺部疾病的唯一治疗方法。我们对自1992年丹麦启动国家肺移植项目以来接受移植的CF患者的结局进行了回顾性研究。
在10年期间，47例CF患者被列入肺移植名单；29例患者接受了移植，18例患者在等待供体器官时死亡。11例患者接受了整块双肺移植并直接进行支气管动脉血运重建，18例患者接受了双侧序贯肺移植。移植时的中位年龄为29岁（范围11 - 50岁）。
围手术期死亡率（≤30天）为3.5%（1/29例患者）。移植患者1年、3年、5年和8年的精算生存率分别为89%、80%、80%和70%。等待名单上未移植患者1年和2年的精算生存率分别为28%和11%（P<0.0001）。移植患者的死亡原因如下：第7天呼吸衰竭（n = 1）、闭塞性细支气管炎综合征（n = 2）、感染（巨细胞病毒、烟曲霉）（n = 2）、支气管吻合口裂开（n = 1）。肺功能（预测FEV1%）从移植前的中位20%（范围13 - 31）改善到5年后的71%（范围19 - 118）（P<0.0001）。肾功能（51Cr - EDTA清除率）从移植前的中位97 ml/min（范围45 - 190）降至移植后6个月的32 ml/min（范围8 - 84）（P<0.001）。3例患者（11%）移植后接受了透析，其中2例接受了肾移植。与达利珠单抗相比，用兔抗胸腺细胞球蛋白进行免疫抑制诱导治疗导致移植后前3个月内急性排斥反应的治疗次数更少（5 - 8周时P = 0.05）。移植前3例患者中存在多食伯克霍尔德菌，其中1例患者获得了满意的长期结局。
肺移植是一种成熟的、可延长CF和终末期肺部疾病患者生命的治疗方法。手术死亡率低，CF患者肺移植后有显著的早期生存获益。在这群年轻且重症的患者中可取得满意的长期结果。
