Lung Transplant Program, Pulmonary, Allergy and Critical Care Medicine Division, University of Pittsburgh Medical Center (UPMC); Cystic Fibrosis Program, UPMC Presbyterian and UPMC Children's Hospital, NW 628 MUH, 3459 Fifth Avenue, Pittsburgh, PA 15213, USA.
Clin Chest Med. 2022 Dec;43(4):821-840. doi: 10.1016/j.ccm.2022.07.002.
Lung transplantation provides a treatment option for many individuals with advanced lung disease due to cystic fibrosis (CF). Since the first transplants for CF in the 1980s, survival has improved and the opportunity for transplant has expanded to include individuals who previously were not considered candidates for transplant. Criteria to be a transplant candidate vary significantly among transplant programs, highlighting that the engagement in more than one transplant program may be necessary. Individuals with highly resistant CF pathogens, malnutrition, osteoporosis, CF liver disease, and other comorbidities may be suitable candidates for lung transplant, or if needed, multi-organ transplant. The transplant process involves several phases, from discussion of prognosis and referral to a transplant center, to transplant evaluation, to listing, transplant surgery, and care after transplant. While the availability of highly effective CF transmembrane conductance regulator (CFTR) modulators for many individuals with CF has improved lung function and slowed progression to respiratory failure, early discussion regarding transplant as a treatment option and referral to a transplant program are critical to maximizing opportunity and optimizing patient and family experience. The decision to be evaluated for transplant and to list for transplant are distinct, and early referral may provide a treatment option that can be urgently executed if needed. Survival after transplant for CF is improving, to a median survival of approximately 10 years, and most transplant survivors enjoy significant improvement in quality of life.
肺移植为许多患有囊性纤维化 (CF) 等晚期肺部疾病的患者提供了一种治疗选择。自 20 世纪 80 年代首例 CF 肺移植以来，患者的存活率得到了提高，并且移植机会也扩大到了以前不符合移植条件的患者。不同的移植项目对移植候选人的标准差异很大，这表明患者可能需要参与多个移植项目。患有高度耐药 CF 病原体、营养不良、骨质疏松症、CF 肝疾病和其他合并症的患者可能适合进行肺移植，或者如果需要，也可以进行多器官移植。移植过程涉及多个阶段，包括讨论预后和转诊至移植中心、移植评估、登记、移植手术以及移植后的护理。虽然对于许多 CF 患者来说，高效的 CF 跨膜电导调节剂 (CFTR) 调节剂的出现改善了肺功能并减缓了呼吸衰竭的进展，但早期讨论将移植作为一种治疗选择并转诊至移植项目对于最大限度地提高机会和优化患者及其家属的体验至关重要。评估移植和登记移植是两个不同的决策，早期转诊可能提供一种可以在需要时紧急执行的治疗选择。CF 患者的移植后存活率正在提高，中位数约为 10 年，大多数移植幸存者的生活质量都有显著改善。
