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Purpose: To investigate how revision of the organ transplant law in Japan affected lung transplantation in this country., Methods: Lung transplant candidates registered between January, 2000 and December, 2009 were designated as the pre-revision group (n = 396) and those registered between January, 2011 and December, 2020, as the post-revision group (n = 1326). Both groups were analyzed retrospectively using data collected by the Japanese Society of Lung and Heart-Lung Transplantation., Results: The number of patients who underwent brain-dead donor lung transplantation (BDLT) increased significantly after the law amendment (32.2 vs. 13.8%, p < 0.01). The median waiting time for BDLT was significantly reduced (708 days vs. 1163 days, p < 0.01) and the mortality rate while waiting for BDLT improved significantly after the law amendment (33.1 vs. 42.6%, p < 0.01). In the post-revision group, 18 pediatric patients underwent BDLT. The 5-year survival rates after BDLT were comparable between the groups (73.5% in the pre-revision group vs. 73.2% in the post-revision group, p = 0.32)., Conclusions: The organ transplant law revision shortened the waiting time for BDLT significantly and decreased the mortality rate while waiting for BDLT. The posttransplant outcomes in Japan remained favorable throughout the study period., (© 2024. The Author(s) under exclusive licence to Springer Nature Singapore Pte Ltd.)

Background: Whether comprehensive risk assessment predicts post-referral outcome in patients with pulmonary arterial hypertension (PAH) referred for lung transplantation (LT) in Japan is unknown., Methods and Results: We retrospectively analyzed 52 PAH patients referred for LT. Risk status at referral was assessed using 3- and 4-strata models from the 2022 European Society of Cardiology and European Respiratory Society guidelines. The 3-strata model intermediate-risk group was further divided into 2 groups based on the median proportion of low-risk variables (modified risk assessment [MRA]). The primary outcome was post-referral mortality. During follow-up, 9 patients died and 13 patients underwent LT. There was no survival difference among 3-strata model groups. The 4-strata model classified 33, 16, and 3 patients as low intermediate, high intermediate, and high risk, respectively. The 4-strata model identified high-risk patients with a 1-year survival rate of 33%, but did not discriminate survival between the intermediate-risk groups. The MRA classified 15, 28, 8, and 1 patients as low, low intermediate, high intermediate, and high risk, respectively. High intermediate- or high-risk patients had worse survival (P<0.001), with 1- and 3-year survival rates of 64% and 34%, respectively. MRA high intermediate- or high-risk classification was associated with mortality (hazard ratio 12.780; 95% confidence interval 2.583-63.221; P=0.002)., Conclusions: Patients classified as high intermediate or high risk by the MRA after treatment should be referred for LT.

Background: This retrospective observational study aimed to examine whether clinical inflammatory parameters were associated with the requirement dosage of unfractionated heparin (UFH) to maintain the range of ACT in veno-arterial extracorporeal membrane oxygenation (V-A ECMO) during lung transplantation surgery., Methods: Among all patients who underwent lung transplantation using V-A ECMO from January 2021 to May 2022, 27 patients were included. These patients were divided into two groups based on whether the infusion rate of UFH was increased from the initial infusion rate (7-8 units/kg/h) (increased group, n = 10) or the infusion rate was maintained or decreased (non-increased group, n = 17). The infusion rate was adjusted with an activated clotting time (ACT) target of 160-200 s., Results: At 1-2 h after starting ECMO, ACT was significantly lower (179.0 (166.5-188.5) versus 224.0 (193.0-242.0) sec, p = 0.006) and white blood cell (WBC) counts were higher in the increased group (12.6 ± 3.3 versus 9.5 ± 4.0 × 10 3 /μL, p = 0.046). The UFH infusion rates were higher in the increased group during the surgery. The cutoff value of WBC count at 1-2 h after starting ECMO for discriminating the need for increasing the UFH dosage was determined as 10.2 × 10 3 /μL (sensitivity 90.0%, specificity 58.8%, area under the curve 0.712) and discrimination of this cut-off value was confirmed as statistically significant (p = 0.018)., Conclusion: These data suggested that WBC count was associated with the requirement of an increase in the UFH infusion rate of V-A ECMO during lung transplantation surgery. Further evaluation is necessary to clarify the role of WBC count in determining the optimal UFH dosage., (© The Author(s), published by EDP Sciences, 2024.)

Macrophages contribute to post-transplant lung rejection. Disulfiram (DSF), an anti-alcoholic drug, has an anti-inflammatory effect and regulates macrophage chemotactic activity. Here, we investigated DSF efficacy in suppressing acute rejection post-lung transplantation. Male Lewis rats (280-300 g) received orthotopic left lung transplants from Fisher 344 rats (minor histocompatibility antigen-mismatched transplantation). DSF (0.75 mg/h) monotherapy or co-solvent only (50% hydroxypropyl-β-cyclodextrin) as control was subcutaneously administered for 7 days (n = 10/group). No post-transplant immunosuppressant was administered. Grades of acute rejection, infiltration of immune cells positive for CD68, CD3, or CD79a, and gene expression of monocyte chemoattractant protein and pro-inflammatory cytokines in the grafts were assessed 7 days post-transplantation. The DSF-treated group had significantly milder lymphocytic bronchiolitis than the control group. The infiltration levels of CD68 + or CD3 + cells to the peribronchial area were significantly lower in the DSF than in the control groups. The normalized expression of chemokine ligand 2 and interleukin-6 mRNA in allografts was lower in the DSF than in the control groups. Validation assay revealed interleukin-6 expression to be significantly lower in the DSF than in the control groups. DSF can alleviate acute rejection post-lung transplantation by reducing macrophage accumulation around peripheral bronchi and suppressing pro-inflammatory cytokine expression., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Yoshiyasu, Matsuki, Sato, Urushiyama, Toda, Terasaki, Suzuki, Shinozaki-Ushiku, Terashima and Nakajima.)

Purpose: To clarify the impact of donor and recipient characteristics on the survival of recipients before and after lung transplantation in the Japanese population., Methods: Patients' data were collected for retrospective analysis from all authorized lung transplant centers in Japan. We included 1963 patients listed for lung transplantation by the end of December 2021, comprised of 658 deceased-donor and 270 living-donor lung transplants., Results: Primary disease had a significant impact on the mortality of patients waiting for transplantation. The indications for transplant significantly affected the post-transplant survival rate of deceased-donor lung transplant recipients. The recipient's age also significantly affected the post-transplant survival rate of the deceased-donor and living-donor lung transplant recipients. The recipients of grafts transplanted from donors aged 61 years or older showed a worse post-transplant survival rate (≧60 years old). The survival rate for the combination of a female donor to a male recipient among the deceased-donor lung transplant recipients was the worst among the four combinations., Conclusion: The donor and recipient characteristics significantly impacted the survival of recipients after lung transplantation. The underlying mechanism of the negative impact of the gender mismatch of female donor to male recipient on post-transplant survival needs to be investigated further., (© 2023. The Author(s).)

Lung transplantation (LT) is the only option for patients with pulmonary arterial hypertension (PAH) refractory to maximal medical therapy. However, some patients referred for LT could survive without LT, and its determinants remain unclear. This study aimed to elucidate prognostic factors of severe PAH at the referral time. We retrospectively analyzed 34 patients referred for LT evaluation. The primary outcome was a composite of death or LT. Over a median follow-up period of 2.56 years, eight patients received LT and eight died. Compared with LT-free survival group, pulmonary arterial systolic pressure (PASP) was higher (p = 0.042), and the ratio of tricuspid annular plane systolic excursion (TAPSE) to PASP (TAPSE/PASP) was lower (p = 0.01) in LT or death group. In receiver operating characteristic analysis, the area under the curve was 0.759 (95% confidence interval 0.589-0.929) for TAPSE/PASP to predict primary outcome, and the optimal cut-off value was 0.30 mm/mmHg (sensitivity 0.875 and specificity 0.667). In a multivariate analysis, TAPSE/PASP was independently associated with death or LT. Kaplan-Meier analysis showed a better LT-free survival in patients with TAPSE/PASP ≧0.30 mm/mmHg than in those with < 0.30 mm/mmHg (p = 0.001). Low-level TAPSE/PASP could be a poor prognostic factor in PAH patients referred for LT evaluation., (© 2023. The Author(s).)

In Japan, successful cases of a bridge to lung transplantation (BTT) by extracorporeal membrane oxygenation (ECMO) are rare. We present the case of a man in his thirties, diagnosed with interstitial pneumonia 6 years prior and registered for lung transplant 1 year prior due to disease progression despite treatment. Due to the patient's worsening respiratory failure, he was transferred to our hospital for BTT by ECMO. Since long-term management was expected and pulmonary hypertension was present, veno-arterial (V-A) ECMO was conducted using the right atrial blood outflow via the right internal jugular vein and right axillary artery inflow via a vascular graft. After tracheostomy, he was managed as "Awake ECMO". In addition, interprofessional collaboration such as physiotherapist rehabilitation, nurses, and liaison teams prevented muscle weakness and supported the mental aspect. We were able to minimize complications such as severe infections and bleeding. A compatible brain-dead donor was found on day 108 after introducing ECMO, and the patient was transferred to a transplant facility on day 109. The peripheral upper V-A ECMO is one of the configurations suitable for long-term BTT management., (© 2022. The Japanese Society for Artificial Organs.)

A 40-year-old woman with idiopathic pleuroparenchymal fibroelastosis (IPPFE) and flat chest underwent left single lung transplantation (SLT). Although she had developed over-systemic pulmonary arterial pressure (PAP) at transplantation, it was alleviated. However, her PAP gradually increased again. Her transplanted lung was well-inflated, but progression of fibrosis in her right native lung appeared to have caused a mediastinal shift, and her flat chest caused obstruction of the outflow tract of the pulmonary vein. She died of heart failure and associated infection 1.5 years after transplantation. An autopsy confirmed irreversible pulmonary arterial and venous changes in the transplanted lung, suggestive of chronic pressure overload. The flat chest associated with IPPFE can affect pulmonary circulation after SLT.

Objectives: Even after transplantation of favourable donor lungs, some recipients require prolonged weaning from mechanical ventilation, indicating a poor prognosis. We investigated the effects of prolonged mechanical ventilation (PMV) for >14 days on the recovery and survival of patients who underwent cadaveric lung transplantation in relation to their physical traits., Methods: We retrospectively reviewed patients who underwent cadaveric lung transplantation (age ≥15 years) at a single centre between April 2015 and December 2020 and classified them into PMV and non-PMV groups (>14 and ≤14 days of mechanical ventilation postoperatively, respectively). The factors predicting PMV comprised clinical factors (e.g. marginal donor) and physical features, namely flat chest, narrow fourth intercostal space (length, <5 mm), mediastinal shift, thoracic mediastinal-occupying ratio (TMOR) >40% and sarcopenia, according to the logistic regression analysis. The log-rank test was used to examine the association between TMOR >40% and 3-year prognosis., Results: The PMV group comprised 17 (33%) of 51 recipients. Multivariable logistic analysis showed that the TMOR >40% (odds ratio, 7.3; 95% confidence interval, 1.3-40.1; P = 0.023) was an independent preoperative predictive factor for PMV postoperatively. Stepwise analysis revealed intraoperative extracorporeal membrane oxygenation and reoperation as postoperative predictive factors in addition to TMOR >40%. Recipients with TMOR >40% had significantly worse 3-year survival than other recipients (71.2% vs 100.0%, respectively; P = 0.008)., Conclusions: Recipients with a TMOR >40% may be long-term ventilator dependent and have a poor prognosis., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Background: Standardized uptake values (SUVs) derived from 18 F-fluorodeoxyglucose ( 18 F-FDG) positron emission tomography (PET) are valuable but insufficient for detecting lung allograft rejection (AR). Using a rat lung transplantation (LTx) model, we investigated correlations of AR with the SUV max and PET-derived radiomics and further evaluated the performance of machine learning (ML)-based radiomics for monitoring AR., Methods: LTx was performed on 4 groups of rats: isograft, allograft-cyclosporine continuous (CsA cont ), allograft-CsA delayed , and allograft-CsA 1week . Each rat underwent 18 F-FDG PET at week 3 or 6. The SUV max and radiomic features were extracted from the PET images. Least absolute shrinkage and selection operator regression was used to construct a radiomics score (Rad-score). Ten modeling algorithms with 7 feature selection methods were performed to develop 70 radiomics models (49 ML models and 21 logistic regression models) for monitoring AR, validated using the bootstrap method., Results: In total, 837 radiomic features were extracted from each PET image. The SUV max and Rad-score showed significant positive correlations with histopathology (p < .05). The area under the curve (AUC) of SUV max for detecting AR was 0.783. The median AUC of ML models was 0.921, which was superior to that of logistic regression models (median AUC, 0.721). The optimal ML model using a random forest modeling algorithm with random forest feature selection method exhibited the highest AUC of 0.982 (95% confidence interval, 0.875-1.000) in all models., Conclusions: SUV max provided a good correlation with AR, but ML-based PET radiomics further strengthened the power of 18 F-FDG PET functional imaging for monitoring AR in LTx., (Copyright © 2022 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Multiple situations necessitate extracorporeal membrane oxygenation( ECMO) during lung transplantation. ECMO can also be used as a bridge to lung transplantation. We describe five cases in which bridging ECMO was successfully utilized, including three cases of living donor lung transplantation. ECMO can also be used as an alternative to cardiopulmonary bypass for intraoperative support during lung transplantation, and postoperatively, primary graft dysfunction, rejection, and infection can cause reversible respiratory failure which warrants ECMO. It can also be used for patients with pulmonary arterial hypertension in the early postoperative period to help their hearts adjust to new circulation. Eight patients with pulmonary arterial hypertension who underwent lung transplantation at our institu-tion received intraoperative and early postoperative ECMO support and their postoperative courses were uneventful. In this report, we review the indications for ECMO and the type of ECMO required for each of the various issues that can arise during lung transplantation, based on the literature and our experiences.

Living-donor lobar lung transplantation is often indicated for acute exacerbation of idiopathic interstitial pneumonia because of the long waiting time for cadaveric lung transplantation in Japan. Donors without major underlying diseases are selected after medical screening. A 44-year-old man donated his right lower lobe to his sibling with idiopathic interstitial pneumonia. Although he was free of any major medical problems before transplantation, fibrotic changes appeared in both the donated lung and the donor's remaining lungs in a case of familial interstitial pneumonia. For living-donor lobar lung transplantation for idiopathic interstitial pneumonia, donor candidates should be informed of the potential issue of a familial disease., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Currently, inhaled nitric oxide (NO) therapy for lung transplantation is not covered by public health insurance in Japan. In this study, we evaluated the perioperative use and safety of inhaled NO therapy for lung transplantation., Methods: Data regarding the duration of treatment and adverse events of inhaled NO therapy were collected for all lung transplantations performed from January 1, 2015, to December 31, 2019, at nine lung transplant facilities in Japan., Results: During the study period, lung transplants were performed in 357 patients, among whom inhaled NO therapy was administered to 349 patients (98%). The median initial and median maximum inhaled NO doses were 10 and 20 ppm, respectively. Inhaled NO therapy was introduced during surgery and continued postoperatively in 313 patients (90%) for a median of 4 days. Significant improvements in oxygenation and decreases in pulmonary arterial pressure were observed in patients receiving inhaled NO therapy. Side effects of inhaled NO therapy, such as methemoglobinemia, were observed in 15 patients (4%), with a significant incidence in patients aged < 18 years., Conclusions: Inhaled NO therapy was performed in almost all patients who underwent lung transplantation in Japan and showed reasonable efficacy. Therefore, public health insurance coverage for inhaled NO therapy during lung transplantation is recommended., (© 2021. The Japanese Association for Thoracic Surgery.)

Background: Although the number of patients who undergo extracorporeal membrane oxygenation (ECMO) as a bridge to lung transplantation is increasing worldwide, there are few reports on lung transplantation after long-term ECMO (more than 1 month). We report a rare case of successful bilateral lung transplantation in a Japanese patient after 5 months of veno-venous (VV)-ECMO support., Case Presentation: A 27-year-old man who underwent bone marrow transplantation (BMTx) with fully matched human leukocyte antigen typing was diagnosed with bronchiolitis obliterans caused by chronic graft-versus-host disease 3 years after the BMTx. One year later, his respiratory condition had exacerbated, with carbon dioxide retention that required conventional mechanical ventilation. He was then deemed a suitable candidate for lung transplantation by a multidisciplinary transplantation selection committee. While waiting for donor lungs, his hypercapnia and acidosis became barely manageable under care with mechanical ventilation and ultimately he was switched to VV-ECMO. He remained on VV-ECMO for the next 5 months, during which period the circuit was switched nine times. In addition, sophisticated intensive care was required to manage multiple episodes of sepsis and coagulopathy. A suitable donor was identified 5 months later, and bilateral lung transplantation was initiated with continuous VV-ECMO. The procedure itself was extremely challenging owing to severe adhesions resulting from previous thoracotomy, inflammation, infection, and intrapulmonary hemorrhage. The operative time for the transplantation was about 19 h. Currently, at 2 years 8 months postoperatively, the patient is alive and well., Conclusion: Transplant surgery in this patient was extremely challenging because of the presence of severe pleural adhesions and stiff native lungs secondary to hemorrhagic complications due to the prolonged ECMO support. Surgeons must recognize that lung transplantation after long-term ECMO bridging can be technically more complicated and challenging than shorter-term ECMO., (© 2021. The Author(s).)

Purpose: This study was performed to compare the outcome of lung transplantation (LT) for idiopathic pleuroparenchymal fibroelastosis (IPPFE) with that of LT for idiopathic pulmonary fibrosis (IPF)., Methods: We reviewed, retrospectively, all adult patients who underwent LT for IPPFE or IPF in Japan between 1998 and 2018., Results: There were 100 patients eligible for this study (31 with IPPFE and 69 with IPF). Patients with IPPFE tended to have a significantly lower body mass index (BMI) than those with IPF (median, 16.7 vs. 22.6 kg/m 2 , respectively; P < 0.01). However, Kaplan-Meier survival curves showed no significant difference in overall survival between the groups. The BMI did not increase in patients with IPPFE, even 1 year after LT (pretransplant, 16.5 ± 3.2 kg/m 2 vs. 1 year post-transplant, 15.6 ± 2.5 kg/m 2 ; P = 0.08). The percent predicted forced vital capacity (%FVC) 1 year after LT was significantly lower in the IPPFE group than in the IPF group (48.4% ± 19.5% vs. 68.6% ± 15.5%, respectively; P < 0.01)., Conclusions: Despite extrapulmonary problems such as a flat chest, low BMI, and associated restrictive impairment persisting in patients with IPPFE, patient survival after LT for IPPFE or IPF was equivalent., (© 2021. Springer Nature Singapore Pte Ltd.)

Lung transplantation (LTx) and lung volume reduction surgery are established therapies for end-stage chronic obstructive pulmonary disease. Although native lung hyperinflation is a well-known complication of unilateral LTx for chronic obstructive pulmonary disease, the unilateral procedure continues to be performed because of severe shortages of cadaveric donors. As native lung hyperinflation can adversely affect the graft, all possible protection should be provided for patients with one-lobe transplantation. We report an emphysematous juvenile patient who successfully underwent simultaneous living-donor, single-lobe LTx and volume reduction in the contralateral lung., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The use of bioartificial lungs may represent a breakthrough for the treatment of end-stage lung disease. The present study aimed to evaluate the feasibility of transplanting bioengineered lungs created from autologous cells. Porcine decellularized lung scaffolds were seeded with porcine recipient-derived airway and vascular cells. The porcine recipient-derived cells were collected from lung tissue obtained by pulmonary wedge resection. Following culture of autologous cells in the scaffolds, the resulting grafts were unilaterally transplanted into porcine recipients (n = 3). Allograft left unilateral lung transplantation was performed in the control group (n = 3). Left unilateral transplantation of decellularized grafts was also performed in a separate control group (n = 2). In vivo functions were assessed for 2 hours after transplantation. Histologic evaluation and immunostaining showed the presence of airway and vascular cells in the bioengineered lungs. No animals survived in the decellularized transplant group, whereas all animals survived in the bioengineered transplant and allotransplant groups. However, bioengineered lung grafts showed marked bullous changes. The oxygen exchange was comparable between the bioengineered lung graft transplant and allograft transplant groups. However, the carbon dioxide gas exchange of the bioengineered lung graft transplant group was significantly lower than that of the allograft transplant group at 2 hours after transplantation (4.10 ± 0.87 mm Hg vs 24.7 ± 10.1 mm Hg, P = 0.02). Transplantation of bioartificial lung grafts created from autologous cells was feasible in the super-acute phase. However, bullous changes and poor carbon dioxide gas exchange remain limitations of this method., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Ex vivo lung perfusion (EVLP) is reportedly a useful strategy that permits marginal donor lungs to be evaluated and reconditioned for successful lung transplantation (LTx). This systematic review and meta-analysis was performed to evaluate the outcomes of EVLP conducted for marginal donor lungs., Methods: We searched PubMed, the Cochrane Library, and Embase to select studies describing the results of LTx following EVLP for marginal donor lungs compared with standard LTx without EVLP. We performed a meta-analysis to examine donor baseline characteristics, recipient baseline characteristics, and postoperative outcomes., Results: Of 1380 studies, 8 studies involving 1191 patients met the inclusion criteria. Compared with the non-EVLP group (ie, standard LTx without EVLP), the EVLP group (ie, EVLP of marginal donors following LTx) had similar donor age and sex and recipient baseline age, sex, body mass index, bridge by ventilator/extracorporeal life support/extracorporeal membrane oxygenation, and rate of double LTx but more abnormal donor lung radiographs (P = .0002), a higher smoking history rate (P = .03), and worse donor arterial oxygen tension/inspired oxygen fraction (P < .00001). However, there were no significant differences in outcomes between the EVLP and non-EVLP groups with respect to the length of postoperative intubation, postoperative extracorporeal life support/extracorporeal membrane oxygenation use, length of intensive care unit stay, length of hospital stay, 72-hour primary graft dysfunction of grade 3, 30-day survival, or 1-year survival (all P values > .05)., Conclusions: Posttransplant outcomes were similar between EVLP-treated LTx and standard LTx without EVLP, although the quality of donor lungs was worse with EVLP-treated LTx., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Introduction: The prognostic implications of having patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE) on lung transplantation waiting lists have been unclear. In Japan, where a severe shortage of brain-dead donors remains a major limitation for organ transplantation, it is particularly important to predict the prognoses of patients when they are listed for transplantation. The purpose of this study was to investigate the characteristics of lung transplantation candidates with IPPFE and the influence of those characteristics on prognosis., Methods: This was a retrospective review of 29 consecutive adult lung transplant candidates with idiopathic interstitial pneumonia between January 2014 and April 2018., Results: Eight patients with IPPFE and 21 with other types of idiopathic interstitial pneumonia were included. Body mass index (median 17.1 kg/m 2 vs 23.5 kg/m 2 , P < .01) and ratio of anteroposterior to transverse diameter of the thoracic cage were significantly lower (0.530 vs 0.583, P = .02) in the IPPFE group. Patients with a body mass index <20.0 kg/m 2 (P = .02), 6-minute walk distance <250.0 m (P < .01), ratio of PaO 2 to fraction of inspiratory oxygen <300.0 mm Hg (P < .01), and an inability to perform the diffusing capacity of carbon monoxide test (P < .01) had significantly shorter survival times in the other idiopathic interstitial pneumonia, but not in the IPPFE, group. Some patients with IPPFE survived for long enough to undergo transplantation., Conclusions: Patients with IPPFE waiting for transplantation have some distinctive characteristics and should be retained on waiting lists to receive transplants., (Copyright © 2019. Published by Elsevier Inc.)

A patient with pleuroparenchymal fibroelastosis (PPFE) was successfully treated with living-donor lobar lung transplantation. A 27-year-old woman with a 3-month history of dyspnea received a diagnosis of PPFE. Her chest wall was extremely flattened over time, and her respiratory condition progressively deteriorated. She underwent semielective bilateral living-donor lobar lung transplantation. Her chest wall rigidity, which was secondary to PPFE, required intensive pulmonary rehabilitation postoperatively. By 6 months after transplantation, the flattening of her chest wall was reversed. Living-donor lobar lung transplantation was a life-saving procedure for this patient and improved the chest wall deformity of PPFE., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Primary lung cancer arising in a transplanted lung is much rarer than cancer arising in a native lung. We herein describe a case of lung cancer developing in a transplanted lung after single-lung transplantation. Wedge resection was safely and successfully completed using venovenous extracorporeal membrane oxygenation (VV-ECMO). Case presentation: A 63-year-old man underwent right single-lung transplantation for idiopathic interstitial pneumonia from a donor in his 60s with a 44-pack-year history of smoking. One year 6 months later, computed tomography revealed a 10-mm nodule in the right lower lobe of the transplanted lung. Fluorodeoxyglucose-positron emission tomography showed abnormal accumulation of fluorodeoxyglucose (maximum standardized uptake value, 2.8) in the same area, suggesting lung cancer. However, percutaneous or transbronchial biopsy was technically impossible. Surgical diagnosis and treatment were planned, and VV-ECMO was introduced before wedge resection. Although pleural adhesion and the location of the nodule close to the hilum resulted in a challenging procedure, the operation was completed in 190 min. The final pathological diagnosis was papillary adenocarcinoma. Conclusions: Lung cancer arising in a lung transplanted from a brain-dead donor is rare. Limited lung resection using VV-ECMO is considered a useful option, particularly in cases of single-lung transplantation. [ABSTRACT FROM AUTHOR]

A 16-year-old girl with a genetic diagnosis of cystic fibrosis was referred to us for consideration of lung transplantation. She had been hospitalized repeatedly for pneumonia and pneumothoraxes and her respiratory function had worsened progressively. Although she also had liver cirrhosis, she was considered a candidate for lung transplantation because her liver disease was compensated and only slowly progressive. After bilateral lung transplantation from a brain-dead donor, she developed ascites that was well controlled with diuretics. Otherwise, her post-operative course was uneventful and she was transferred to another hospital for rehabilitation 39 days after lung transplantation. • Patients with cystic fibrosis may have various complications in organs other than the lung, which makes isolated lung transplantation challenging. • We report a case of lung transplantation in a patient with cystic fibrosis complicated with liver cirrhosis secondary to cystic fibrosis-associated liver disease, which generally shows mild progression. • Isolated lung transplantation conducted after careful evaluation of the patient's liver cirrhosis resulted in good post-operative course. [ABSTRACT FROM AUTHOR]