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Purpose of Review: Lung transplantation (LTX) has transformed care for people with cystic fibrosis (pwCF) suffering from advanced cystic fibrosis lung disease (ACFLD), and it has evolved into an accepted therapy for patients with ACFLD across all ages. We review cystic fibrosis as a major indication for LTX, particularly highlighting outcomes including survival, a changing landscape over time, and factors affecting sequelae following LTX in cystic fibrosis., Recent Findings: Although some populations such as those undergoing lung retransplantation exhibit inferior posttransplant outcomes, LTX for pwCF provides an excellent long-term survival that has significantly improved over time, likely due to specialized cystic fibrosis center care and recognition of common comorbidities in pwCF post-LTX. There are gaps in post-LTX outcomes for pwCF, including that identified between Canada and the United States, and that seen in adolescents - both of which are likely multifactorial. In particular, the revolution in cystic fibrosis medical therapy with CFTR modulator therapy has resulted in a dramatic decline in programs performing LTX for cystic fibrosis. How durable this effect will remains to be seen., Summary: Overall, LTX remains a well accepted ultimate therapy option in patients with ACFLD if compatible with the individual's goals of care, offering an improved quality of life and maximization of overall survival., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Smoking remains the leading cause of preventable disease and death in the developed world that kills half of all long-term users. Tobacco use after solid organ transplantation is associated with allograft dysfunction, cancer, and reduced overall survival., Methods: In this single-center, retrospective study, we describe the frequency of tobacco use after lung transplantation (LTx), pretransplant patient characteristics associated with tobacco use, and the safety, efficacy, and outcomes of posttransplant tobacco cessation interventions., Results: Four percent of our LTx cohort resumed tobacco use posttransplant. Chronic obstructive pulmonary disease (P = 0.043), the cessation duration before LTx (P < 0.001), and the packyear-cessation index (PCI) (P < 0.001) were found to be significantly associated with tobacco use posttransplant. A PCI cutoff value of 0.32 had 100% sensitivity and 45% specificity for tobacco use resumption. Thirty-five percent of the posttransplant tobacco users successfully quit tobacco consumption., Conclusions: Patients with chronic obstructive pulmonary disease and a short duration of smoking cessation before LTx were at greatest risk of tobacco use after LTx. The PCI may be a useful predictor of tobacco use resumption. Pharmacological tobacco cessation interventions were found to have a comparable safety and efficacy profile compared to nontransplant patients.

Purpose of Review: Lung transplantation has become an accepted therapy in infants, children, and adolescents suffering from end-stage lung diseases, an impaired quality of life and reduced life expectancy. The aim of this review is to highlight specific aspects of pediatric lung transplantation and to give an update on recent findings., Recent Findings: Currently, over 100 lung transplant procedures are performed in children annually worldwide. Long-term success is limited by availability of donor organs and waitlist mortality pretransplant, and an increased infection risk because of immunosuppression, and most importantly late complications, such as chronic lung allograft dysfunction, medication nonadherence, and transition intricacies., Summary: Specific aspects of pediatric lung transplantation will be reviewed and an update on most recent developments in the management of pediatric lung transplant recipients given.

Background: Extracorporeal life support (ECLS) as a bridge to lung transplantation (LuTx) is a promising option for patients with end-stage lung disease on the transplant waiting list. We investigated the outcome of patients bridged to lung transplantation on ECLS technologies, mainly extracorporeal membrane oxygenation (ECMO)., Methods: Between January 2007 and October 2013, ECLS was implanted in 30 patients with intention to bridge to LuTx. Twenty-six patients (26/30) were successfully bridged to LuTx on ECLS. The most common diagnosis was cystic fibrosis (N = 12). Venovenous ECMO was used in 10, venoarterial in 4, interventional lung assist in 5, and stepwise combination of them in 7 recipients., Results: Two patients weaned from ECMO, and 2 patients died on ECMO on the waiting list. Median duration of ECLS was 21 days (1-81 years). Six patients were awake and spontaneously breathing during ECLS support. Thirty-day, 1-year, and 2-year survivals were 89%, 68%, and 53%, respectively, for bridged patients and 96%, 85%, and 79%, respectively, for control group (P = 0.001). Three months conditional survivals were 89% and 69% at 1 and 2 years for ECLS group, compared to 92% and 86% for control group (P = 0.03). Cystic fibrosis recipients had 82% survival rate at 1 and 2 years. All recipients bridged to LuTx on awake ECLS (N = 6) are alive with a median follow-up of 10.8 months (range, 6-21 months)., Conclusions: Our data show significantly lower survival in this high-risk group compared to patients transplanted without preoperative ECLS. Awake and ambulatory ECLS provides the best prognosis for these high-risk patients.

Purpose of Review: Lung transplantation has evolved as an accepted therapy in carefully selected children with end-stage lung disease, offering a prolonged survival and improved quality of life. Presently, more than 100 lung transplant procedures are done in children annually worldwide. Specific aspects of children and adolescents undergoing lung transplantation will be reviewed and an update on most recent developments in the management of paediatric lung transplant recipients will be given., Recent Findings: Certain aspects are unique to children and adolescents undergoing lung transplantation such as a challenging surgical procedure, the effects of immunosuppressant therapy, and the impact of infections on the child's developing immune system and somatic growth. The underlying diagnoses leading to lung transplantation vary considerably by age group. Early referral, careful patient selection and appropriate timing of listing are crucial to achieve the maximal survival benefit. In particular, infectious complications are a common in children. Chronic lung allograft dysfunction remains the major obstacle for a better long-term survival., Summary: Lung transplantation has successfully been performed in infants, children and adolescents with outcomes similar to that in adults. Specific aspects of paediatric lung transplantation include donor shortage for smaller recipients, somatic growth, psychosocial aspects and adherence, and transition to adult care.

Background: The successful use of azithromycin in lung transplant recipients with Bronchiolitis Obliterans Syndrome (BOS) has been described, its mechanisms of action, however, are not known. We report the largest experience of long-term clarithromycin (CLAR) in lung transplant patients with BOS or potential BOS., Methods: Transplant recipients receiving CLAR for BOS or potential BOS were included (2000-2008). Response to CLAR was defined as an FEV1 increase of >10% at 6 months compared to the FEV1 recorded at initiation of CLAR., Results: 31 patients were included. 12 recipients (39%) responded to CLAR after 6 months, 10/12 recipients responded already after 3 months. Responders showed a mean FEV1 increase of 732 mL (95% CI: 537-938 mL) by 6 months (p<0.001). Overall, patients experienced a FEV1 decline of 181 mL/month prior to CLAR, and a 25 mL/month decline over the 6-months after initiation of CLAR; p=0.015, mean (95% CI) change in rate of decline 155 (32-279)., Discussion: We show that long-term CLAR effectively improves lung function in more than one-third of recipients with BOS or potential BOS, comparable to results reported with long-term azithromycin.

We report the largest single-center experience with extracorporeal photopheresis (ECP) for bronchiolitis obliterans syndrome (BOS) and recurrent acute rejection (AR) after lung transplantation. Lung transplant recipients undergoing ECP for BOS and recurrent AR were included (1997-2007). The rate of forced expiratory volume in 1 second (FEV1) decline was used as the primary measure and graft survival post-ECP as the secondary measure of efficacy. Twenty-four transplant recipients were included (BOS, n=12; recurrent AR, n=12). In recipients with BOS, decline in FEV1 was 112 mL/month before the start of ECP and 12 mL/month after 12 ECP cycles (P=0.011), mean (95% CI) change in rate of decline was 100 (28-171). Median patient survival was 7.0 (range, 3.0-13.6) years, median patient survival post-ECP 4.9 (range, 0.5-8.4) years. No ECP-related complications occurred. Extracorporeal photopheresis reduces the rate of lung function decline in recipients with BOS and is well tolerated. Furthermore, recipients with recurrent AR experience clinical stabilization. However, the underlying mechanism of ECP remains subject to further research.