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Objectives: Lung transplantation (LT) is an effective treatment for patients with interstitial lung disease (ILD) refractory to medical treatment. Although the cases of cadaveric LT (CLT) have increased, the donor shortage in Japan has remained severe. This study aimed to evaluate the International Society of Heart and Lung Transplantation (ISHLT) listing criteria for LT in patients with ILD by predicting outcomes during the waiting time for CLT., Methods: We retrospectively identified 166 patients with fibrotic ILDs who were evaluated and registered for CLT at Kyoto Universal Hospital from April 1, 2008, to December 31, 2017. We examined the correlation between individual parameters of the ISHLT listing criteria and patient outcomes., Results: Among 166 patients, 57 (34.3%) underwent CLT, whereas 83 (50.0%) died before CLT. The median survival time from the date of registration was 22.5 months. The 2-year survival rate was 47.8%. On multivariate Cox proportional hazards analysis, relative decline of percent predicted forced vital capacity (%FVC) in 6 months ≥ 10% (hazard ratio [HR]: 1.72; 95% confidence interval [95%CI]: 1.03-2.87, p = 0.04) and 6-min walking distance (6MWD) < 250 m (HR: 2.77; 95%CI: 1.64-4.69, p < 0.001) were independently associated with worse outcome (i.e., death or living-donor lobar LT)., Conclusions: The 2014 ISHLT criteria could appropriately identify patients with ILD who have a potentially poor prognosis. In particular, 6-month decline in %FVC and shorter 6 min walk distance may be useful for selecting patients with higher risks of mortality., (© 2022. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.)

We report our successful experience in two lung transplant cases in which ex vivo lung perfusion (EVLP) was used to evaluate the function of injured brain-dead donor lungs that were otherwise initially unacceptable. After the donor's lungs were declined for transplantation by all other transplant centers, the lungs were offered to the patients listed for lung transplantation in our hospital. The donor lung function was considered acceptable for transplantation after the 3-h EVLP assessment. In the first case, a 32-year-old man with bronchiolitis obliterans after hematopoietic stem cell transplantation underwent hybrid lung transplantation, that was right brain-dead donor lung transplantation, combined with native-upper lobe sparing living-donor lobar lung transplantation on the left side. In the second case, a 61-year-old woman received the right single lung transplantation for idiopathic pulmonary fibrosis. Both patients are doing well at one and a half years after lung transplantation., (© 2022. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.)

This report describes 2 cases of dyskeratosis congenita in patients with progressive pulmonary diseases who required urgent living-donor lung transplantations. In the first case, a 13-year-old boy underwent bone marrow transplantation for aplastic anemia at age of 6 years. Severe pulmonary right-to-left shunting and fibrosis developed, and his condition deteriorated with acute fibrosis exacerbation and increased intrapulmonary shunt. He underwent lung transplantation and recovered uneventfully. In the second case, a 3-year-old girl with Revesz syndrome underwent bone marrow transplantation for refractory cytopenia. At 6 years of age, she had progressive hypoxia and a brain abscess. Her respiratory condition worsened, and she recovered uneventfully after urgent lung transplantation., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)