Lung Transplantation For Fibrosis Research Articles

Acute worsening of native lung fibrosis after single lung transplantation for pulmonary fibrosis: two case reports

BackgroundIn patients receiving single lung transplantation for idiopathic pulmonary fibrosis, worsening of fibrosis of the native lung is usually progressive over time, with no significant effects on gas exchange.Case presentationHere, we describe the cases of two Caucasian male recipients of single lung transplants for idiopathic pulmonary fibrosis, 65 and 62 years of age, who exhibited acute worsening of lung fibrosis after an episode of serious viral infection (cytomegalovirus primo-infection in one case and COVID-19 in the other). In both cases, along with opacification of the native lung over several days, the patients presented acute respiratory failure that required the use of high-flow nasal oxygen therapy. Eventually, hypoxemic respiratory failure resolved, but with rapid progression of fibrosis of the native lung.ConclusionWe conclude that acute worsening of fibrosis on the native lung secondary to a severe viral infection should be added to the list of potential complications developing on the native lung after single lung transplantation for idiopathic pulmonary fibrosis.

Adult Patients Affected by Cystic Fibrosis in Therapy with Cystic Fibrosis Transmembrane Regulator Modulators and Lung Transplant: Renal Function, Metabolic and Nutritional Status

Background. Cystic fibrosis (CF) is one of the most frequent genetic diseases. The discovery and implementation of new therapies prolonged the survival of CF patients in the last years. Evaluation of long-term complications could be useful to improve the outcome of these patients. Aim of the Study. To evaluate renal function, metabolic, nutritional, and inflammatory status in CF patients on cystic fibrosis transmembrane regulator (CFTR) modulators therapy as well as lung transplant recipients (LRs) and patients on conservative therapy (control group). Materials and Methods. We performed a prospective, longitudinal study on 69 CF patients. Clinical and laboratory parameters (metabolic and nutritional indices and inflammatory markers) were evaluated in all patients before starting CFTR therapy or transplant (T0) and after 3 years (T1). Results. We enrolled 69 CF patients (42 males). Patients were distributed into three groups. The average age was 35.01 ± 10.57 years for the control group (group 0), 32.47 ± 9.40 years for patients on CFTR modulators therapy (group 1), and 38.93 ± 7.14 years for LRs (group 2). At T1, we showed a significant difference among the three groups in terms of renal function indices: creatinine, eGFR, serum nitrogen as well as serum uric acid, sodium, and potassium ( p < 0.001 , p < 0.001 , p < 0.001 , p < 0.001 , p < 0.001 , and p < 0.001 , respectively), particularly in LRs patients. Significant differences were found in nutritional status parameters among the three groups: total protein, serum albumin, serum fibrinogen, serum transferrin, and white blood cell counts ( p < 0.001 , p = 0.037 , p = 0.04 , p = 0.003 , and p = 0.007 , respectively), particularly in LRs compared with other groups. Moreover, we found significant differences in metabolic profile (HbA1c, p = 0.026 ) and inflammatory status, with a significant difference in C-reactive protein values, neutrophil counts, and neutrophil-lymphocyte ratio (NLR) among the three groups ( p < 0.001 , p = 0.005 , and p = 0.026 , respectively). Conclusions. Our study showed a reduced renal function and poor nutritional status in LRs, along with worse metabolic control. Moreover, we showed a lower inflammatory status in patients on CFTR modulators therapy. Therefore, we suggest early and careful monitoring of renal function, metabolic, and nutritional parameters in CF patients, whether they are on conservative therapy, CFTR modulators therapy, and LRs patients.

Fungki Ulcers

A 61-year-old female with a history of bilateral lung transplantation for idiopathic pulmonary fibrosis presented with shortness of breath, cough and diarrhea. She was on chronic immunosuppressive medications, including Tacrolimus and mycophenolate, and on apixaban for deep vein thrombosis.

Effect of pirfenidone on wound healing in lung transplant patients

Background: The drug pirfenidone has been shown to slow the progression and decrease mortality of idiopathic pulmonary fibrosis (IPF). Its exact mechanism is unknown, but it likely inhibits pro-fibrotic cytokine transforming growth factor beta, a known contributor to wound healing. We evaluated whether patients taking pirfenidone until lung transplantation had increased risk of impaired wound healing post-transplant. This information could determine whether pirfenidone should be discontinued prior to listing to allow for a wash-out period. Methods: We retrospectively reviewed patients who underwent lung transplantation for pulmonary fibrosis at Norton Thoracic Institute in Phoenix, Arizona, from January 2014 to December 2015. Results: We describe 18 patients who took pirfenidone up to a month before transplant. Aside from one patient who experienced sternal dehiscence due to a surgical issue, all remaining patients did well with no evidence of airway dehiscence. Each of these 17 patients had been on pirfenidone for at least 30 days; nine patients had been on pirfenidone for over 90 days. Baseline characteristics including age, sex, body mass index, renal function, liver function, glucose level, pre-transplant corticosteroid use, and post-transplant immunosuppressant therapy were similar. Conclusions: In our experience, pirfenidone may be safely continued until lung transplantation. Only one patient in our series experienced impaired wound healing related to a surgical issue, even when pirfenidone was continued until lung transplantation. We found no evidence of impaired wound healing or airway complications after lung transplantation in patients who were treated with pirfenidone before lung transplantation.

Bigger is Better - Oversizing May Improve Survival in Single Lung Transplantation for Pulmonary Fibrosis

Purpose The donor to recipient predicted total lung capacity (pTLC) ratio is routinely used to guide size matching. Guidelines do not differentiate between underlying diseases. We aimed to assess if post-transplant outcomes for pulmonary fibrosis (PF) single lung transplant recipients is improved when receiving an oversized lung. Methods Data on all adult patients who received a first single or bilateral lung-only transplant in the UK between 2007 and 2016 were obtained from the UK Transplant Registry. Donor to recipient pTLC mismatch ratio was used to assign patients to three groups: 0%. Post-transplant survival was assessed using Kaplan-Meier survival curves and log-rank tests. The pTLC mismatch ratio was further analysed as a continuous non-linear variable using an unadjusted Cox Proportional Hazards Regression model. A natural cubic spline was used to represent non-linearity, enabling cubic expressions between “knots” at the 5%, 35%, 65% and 95% percentiles. Results 321 patients were included in our cohort; 243 (75.7%) were male, mean age of 56 years. Mean donor pTLC was 5.7L, mean recipient pTLC was 6.4L, mean pTLC mismatch ratio was -10.1%. Kaplan-Meier analysis suggested no significant difference between the mismatch categories upon 90 day (p=0.28) or 1 year (p=0.16) post-transplant survival, with borderline significance 5 years post-transplant (p=0.08). No significant differences were found analysing the bilateral lung transplant cohort alone. Analysis of single lung recipients showed borderline significance at 1 year (p=0.07) and significant difference 5 years post-transplant (p=0.05). An unadjusted Cox Regression model was therefore applied to single lung transplant recipients only, demonstrating a significant non-linear effect of the pTLC mismatch ratio at 90 days and borderline effect at 1 year and 5 years post transplant (p=0.05, p=0.09 and p=0.06, respectively). The non-linear effect detected for 90 day post-transplant survival was such that the hazard of death decreases the larger the mismatch, until approximately +/-20%. A similar effect was detected for 1 year and 5 years post-transplant, but to a lesser extent. Conclusion Oversizing donor lung allografts in patients receiving a single lung transplant for pulmonary fibrosis may improve survival in both the short and long term.

Need for and Outcomes of Intraoperative Extracorporeal Circulatory Support in Lung Transplantation for Pulmonary Fibrosis

Purpose V-a extracorporeal membrane oxygenation (ECMO) has been successfully used for intraoperative cardiopulmonary support in patients undergoing lung transplantation. Especially patients with lung fibrosis are at risk to require intraoperative v-a ECMO. Aim of this study was to identify predisposing risk factors for intraoperative ECMO support and postoperative outcomes in this cohort of patients. Methods Patients transplanted for lung fibrosis between January 2010 and May 2018 were included into the study. Patients transplanted on v-a ECMO formed group A, the remaining, group B. Follow-up amounted to a mean of 37±27 months. Multivariate forward and backward logistic regression analysis was used to identify risk factors for ECMO. Results Among the 1042 lung-transplanted patients, 313 (30%) patients underwent bilateral transplantation for lung fibrosis. Five patients were excluded as they received conventional cardiopulmonary support due to different resaons. Among the remaining 308 patients, 109 (35%) patients formed group A, 199 (65%), group B. Presence of pulmonary hypertension (OR 3.1; 95%CI 1.8-5.3, p Conclusion Smaller recipient BSA, pulmonary hypertension with high PVR values and evidence of a dilated and hypertrophic right ventricle can predict the need for intraoperative ECMO in patients transplanted for lung fibrosis. Although the postoperative course was more complicated in group A than B, long-term survival did not differ between groups.

Including Cystic-Fibrosis-Specific Variables Improves Accuracy of the Lung Allocation Score

Purpose To identify novel variables associated with waitlist (WL) and posttransplant (PT) mortality for cystic fibrosis (CF) lung transplant patients. To analyze the impact of including new variables in the lung allocation score (LAS) system. Methods The Cystic Fibrosis Foundation Patient Registry (CFFPR) collects data for CF patients prior to listing and the US Scientific Registry of Transplant Recipients Registry collects broad data after listing. These databases were merged by a deterministic algorithm to represent CF patients pre- to posttransplant. The study included candidates and recipients, 1/1/2011-12/31/2014. CFFPR variables known to be important for mortality in CF were considered in Kaplan-Meier univariate time-to-event analyses; significant variables were considered in multivariable Cox proportional hazard regression analysis. LAS and rank, based on models including new variables, were calculated and compared by diagnosis. Results The study cohort consisted of 1020 WL candidates with CF and 677 transplant recipients. Relative drop in FEV1 ≥ 30%, presence of Burkholderia sp., 28-42 days hospitalized, and hemoptysis increased WL mortality. Pulmonary exacerbation time of 15-28 days increased risk of PT mortality. Relative change in FEV1 ≥ 10% increased mortality in chronic obstructive pulmonary disease (COPD) candidates. Including these new variables in the LAS calculation resulted in (1) CF candidates with increased variability in LAS and rank; (2) COPD candidates with higher LAS values and lower rank, indicating increased access to transplant; (3) minimal impact on candidates with other diagnoses. Conclusion Addition of CF-specific variables increased the predictive power of LAS for candidates with CF and COPD. Incorporating CF-specific variables could potentially decrease waitlist mortality for candidates with CF.

Size Matters in Single Lung Transplantation for Pulmonary Fibrosis!

Size Matters in Single Lung Transplantation for Pulmonary Fibrosis!

Myocardial infarction in an adult with cystic fibrosis and heart and lung transplant

We present a case of myocardial infarction in a 19 year old female with cystic fibrosis who had a heart and lung transplant performed at the age of four years old. She presented atypically with a one day history of severe, intermittent, central, sharp chest pain, radiating to her back and down her left arm. A coronary angiogram showed proximal stenosis of the left anterior descending artery and right coronary artery. She was treated with percutaneous coronary intervention, involving drug eluting stents to the left anterior descending artery (LAD) and the right coronary artery (RCA). In this study we discuss the pathophysiology, investigations and treatment of cardiac transplant vasculopathy. Although complete reversal of LAD and RCA stenosis was achieved, routine followup with coronary angiography and careful control of cardiac risk factors will be important to identify and reduce future restenosis and adverse cardiac events.

Preoperative pulmonary artery pressure and mortality after lung transplantation

The purpose of this study was to determine the influence of changes in pulmonary artery pressure during the waiting period on survival after lung transplantation for pulmonary fibrosis. We identified 65 patients with pulmonary fibrosis who underwent lung transplantation from 2003 to 2010. Pulmonary artery pressure determined at listing was compared with intraoperative pressure. The primary outcome was overall survival. Co-variates included type of transplantation (single or bilateral), ischemic time, recipient and donor age and sex. The median age of the 65 patients undergoing transplantation was 58 years, and 27 (43%) underwent bilateral sequential transplantation. Twenty-two (35%) patients presented at transplantation with a mean pulmonary artery pressure increased by at least 10% compared to the initial pressure at the time of listing. Rising pulmonary artery pressure at transplantation was associated with increased mortality (p = 0.022). Other factors including type of operation, ischemic time, age, and sex, were not significantly associated with mortality. Post-transplantation survival was worse among recipients who had pulmonary artery pressure increased by at least 10% at transplantation (p = 0.003, logrank). Increasing pulmonary artery pressure while awaiting lung transplantation is associated with worse long-term survival following transplantation, and is a sign of progressively worsening disease for which greater urgency of donor organ allocation should be considered.

Bilateral versus single lung transplantation for idiopathic pulmonary fibrosis

Bilateral versus single lung transplantation for idiopathic pulmonary fibrosis

711 Changes in Pulmonary Volumes in Patients Who Underwent Lung Transplantation for Pulmonary Fibrosis

711 Changes in Pulmonary Volumes in Patients Who Underwent Lung Transplantation for Pulmonary Fibrosis

Successful treatment of bronchial anastomotic stenosis with modified Dumon Y-stent insertion in lung transplantation: Report of a case

Bronchial complications owing to airway anastomosis still remain a cause of morbidity and mortality following lung transplantation, and bronchial stenosis is the most common manifestation. Current treatment strategies include endoscopic balloon dilation, laser ablation, and stent insertion. Although a variety of stent types are currently available, it is unclear as to which type of prosthesis is most suitable for post-transplant bronchial complications with regard to the primary effects and long-term outcomes. We herein discuss a case of stenosis of the right bronchial anastomosis in a patient who underwent right single lung transplantation for idiopathic pulmonary fibrosis. This complication was successfully treated with the placement of a modified Dumon Y-stent. The stent was removed 2 months after insertion, and the patient has subsequently maintained an adequate airway caliber. Computed tomography, especially the sagittal section through the chest, is useful for detecting bronchial stenosis and monitoring the healing of this condition.

107 Bridge to Lung Transplantation for Pulmonary Fibrosis: From Mechanical Ventilation to Veno-Venous Extracorporeal Membrane Oxygenation with Atrial Septostomy

107 Bridge to Lung Transplantation for Pulmonary Fibrosis: From Mechanical Ventilation to Veno-Venous Extracorporeal Membrane Oxygenation with Atrial Septostomy

447 Improving Survival with Double Lung Transplantation for Pulmonary Fibrosis

447 Improving Survival with Double Lung Transplantation for Pulmonary Fibrosis

Scedosporium colonisation challenges in cystic fibrosis (CF) lung transplantation (LT) – a report of 7 monocentric series

Scedosporium colonisation challenges in cystic fibrosis (CF) lung transplantation (LT) – a report of 7 monocentric series

216: Quality of Life and Functional Outcomes of Patients Undergoing Lung Transplantation for Pulmonary Fibrosis

216: Quality of Life and Functional Outcomes of Patients Undergoing Lung Transplantation for Pulmonary Fibrosis

Impact of Recipient Age and Procedure Type on Survival After Lung Transplantation for Pulmonary Fibrosis

Impact of Recipient Age and Procedure Type on Survival After Lung Transplantation for Pulmonary Fibrosis

La fibrose pulmonaire du syndrome de Hermansky Pudlak n’est pas totalement commune

A 55-year-old female patient with Hermansky-Pudlak syndrome (albinism, thrombopathia and ceroid accumulation) underwent a single lung transplantation for pulmonary fibrosis. Examination of explanted lung showed usual interstitial pneumonia pattern associated with two unusual lesions: presence of numerous macrophages containing ceroid pigments within fibrosis and characteristic foamy swelling of pneumocytes. This later lesion, which has only been recently described, seems related to the mechanism of the disease by defect of surfactant secretion. This lesion allows the histological individualization of pulmonary fibrosis associated with Hermansky-Pudlak syndrome from idiopathic usual interstitial pneumonia.

Impact of pulmonary artery pressure on survival following lung transplantation for idiopathic pulmonary fibrosis

Impact of pulmonary artery pressure on survival following lung transplantation for idiopathic pulmonary fibrosis