Egan T M, Detterbeck F C, Mill M R, Paradowski L J, Lackner R P, Ogden W D, Yankaskas J R, Westerman J H, Thompson J T, Weiner M A
Division of Cardiothoracic Surgery (Department of Surgery), University of North Carolina at Chapel Hill.
J Thorac Cardiovasc Surg. 1995 Feb;109(2):224-34; discussion 234-5. doi: 10.1016/S0022-5223(95)70383-7.
Patients with cystic fibrosis pose particular challenges for lung transplant surgeons. Earlier reports from North American centers suggested that patients with cystic fibrosis were at greater risk for heart-lung or isolated lung transplantation than other patients with end-stage pulmonary disease. During a 3 1/2 year period, 44 patients with end-stage lung disease resulting from cystic fibrosis underwent double lung transplantation at this institution. During the same interval, 18 patients with cystic fibrosis died while waiting for lung transplantation. The ages of the recipients ranged from 8 to 45 years, and mean forced expiratory volume in 1 second was 21% predicted. Seven patients had Pseudomonas cepacia bacteria before transplantation. Bilateral sequential implantation with omentopexy was used in all patients. There were no operative deaths, although two patients required urgent retransplantation because of graft failure. Cardiopulmonary bypass was necessary in six procedures in five patients and was associated with an increased blood transfusion requirement, longer postoperative ventilation, and longer hospital stay. Actuarial survival was 85% at 1 year and 67% at 2 years. Infection was the most common cause of death within 6 months of transplantation (Pseudomonas cepacia pneumonia was the cause of death in two patients), and bronchiolitis obliterans was the most common cause of death after 6 months. Actuarial freedom from development of clinically significant bronchiolitis obliterans was 59% at 2 years. Results of pulmonary function tests improved substantially in survivors, with forced expiratory volume in 1 second averaging 78% predicted 2 years after transplantation. Double lung transplantation can be accomplished with acceptable morbidity and mortality in patients with cystic fibrosis.
囊性纤维化患者给肺移植外科医生带来了特殊的挑战。北美中心早期的报告表明，与其他终末期肺病患者相比，囊性纤维化患者进行心肺联合移植或单肺移植的风险更高。在3年半的时间里，44例因囊性纤维化导致终末期肺病的患者在本机构接受了双肺移植。在同一时期，18例囊性纤维化患者在等待肺移植期间死亡。受者年龄在8至45岁之间，1秒用力呼气量平均为预测值的21%。7例患者在移植前感染洋葱伯克霍尔德菌。所有患者均采用带网膜固定的双侧序贯植入法。尽管有2例患者因移植失败需要紧急再次移植，但无手术死亡病例。5例患者中的6例手术需要体外循环，这与输血需求增加、术后通气时间延长和住院时间延长有关。1年的实际生存率为85%，2年为67%。感染是移植后6个月内最常见的死亡原因（2例患者死于洋葱伯克霍尔德菌肺炎），闭塞性细支气管炎是6个月后最常见的死亡原因。2年时无临床显著性闭塞性细支气管炎发生的实际自由度为59%。肺功能测试结果在存活者中显著改善，移植后2年1秒用力呼气量平均为预测值的78%。对于囊性纤维化患者，双肺移植可以在可接受的发病率和死亡率情况下完成。
