Cadaveric Lung Transplantation Research Articles

Living-donor Lobar Lung Transplantation - Initiation and Development - Secondary Publication.

Due to the difficulty of finding brain-dead donors, in October 1998, I performed the first living-donor lobar lung transplantation (LDLLT) for a ventilator-dependent 24-year-old female patient with bronchiectasis using lobes from her parents. The patient is still alive and in good health 25 years after the transplantation. Over time, the indications for LDLLT have expanded to include pulmonary hypertension, pulmonary fibrosis, congenital genetic diseases, pulmonary complications after stem cell transplantation, and, more recently, severe lung injury due to COVID-19 infection. In 2022, we successfully performed an ABO-incompatible LDLLT. To address size mismatches, we have developed new LDLLT techniques, such as right-to-left inverted transplantation, upper lobe-sparing transplantation, and segmental lung transplantation. Our published studies cover a range of topics, encompassing both basic and clinical research. Of particular significance is the observation that LDLLT offers immunological advantages over cadaveric lung transplantation (CLT). Having conducted 353 cases of lung transplantation, including 161 LDLLTs and 192 CLTs, our 5-year survival rates were 83% after LDLLT and 74% after CLT, surpassing the 55% 5-year survival rate reported by the International Society for Heart and Lung Transplantation. We have hosted numerous observers and research fellows from 15 countries. In addition, I have contributed to LDLLT procedures not only in Japan but also abroad. It brings me great satisfaction to think that my educational efforts may ultimately lead to saving the lives of those suffering from end-stage respiratory failure around the world.

(1420) - Does HLA Mismatch Between Donors and Recipients Influence Postoperative Outcomes in Cadaveric Lung Transplants?

(1420) - Does HLA Mismatch Between Donors and Recipients Influence Postoperative Outcomes in Cadaveric Lung Transplants?

Patient selection, surgery and perioperative management in lung transplantation in Japan.

Lung transplantation (LT) is the final treatment option for end-stage respiratory diseases. The current prognosis of LT recipients in Japan is good, however, the reason for the good prognosis is unclear. In Japan, the waiting time for cadaveric LT is long, which is approximately 900 days on average. A long waiting time affects several aspects of LT. The diseases progress while they await LT in most patients are waiting for LT. Along with the disease progression of the disease, secondary pulmonary hypertension can newly emerge. Some patients suffer from refractory secondary pneumothorax and may receive pleurodesis. Transplant operations can become more difficult, and postoperative management becomes more complicated owing to the disease progression. Thoracic surgeons in Japan have managed the tough difficult situation of LT patients with LT. Possible explanations for how we to maintain a better prognosis in such a situation include sophisticated surgical techniques and ideas, and vigorous postoperative management by thoracic surgeons. Thoracic surgeons are vigorously involved both in operations and in postoperative management in the intensive care unit with or without intensivists in Japan. On the other hand, the long waiting time in Japan and allocation rules with age restriction without considering the severity of patients may have resulted in the selection of recipients to include relatively young recipients, fewer patients with interstitial lung disease and fewer recipients with extracorporeal membrane oxygenation (ECMO) as a bridge to LT. These recipients' characteristics possibly may have affected the prognosis of LT patients with LT in Japan. There is a chance that a future increase in the number of cadaveric donors in Japan may result in a prognosis close that is similar to the international average if the current waiting time in Japan decreases. We review patient selection, surgery and perioperative management in LT in Japan to address the question of why the current prognosis of LT recipients in Japan is good.

Effects of case volume on short- and long-term outcomes following cadaveric lung transplantation in Japan.

Despite the low number of lung transplantations (LTs) in Japan, 10 LT facilities are accredited and good outcomes have been reported. A database review was conducted to clarify the impact of case volume at LT facilities in Japan on short- and long-term outcomes. All cadaveric LT cases treated between 2000 and 2021 in Japan were analyzed using the database of the Japanese Society of Lung and Heart-Lung Transplantation (JSLHT). The nine institutions represented were categorized into the low-volume (LV; <80 cumulative LT cases, <8 LTs/year, n=5) and high-volume (HV; ≥80 cumulative LT cases, ≥8 LTs/year, n=4) centers. Ninety-day and 1-year mortality, as well as 5- and 10-year survival data were evaluated. A total of 658 cadaveric LTs were performed at the nine institutions. The 90-day rates of mortality at the HV and LV centers were 3.5% and 3.9%, respectively (P=0.801), while the 1-year mortality rates were 9.2% and 11.5%, respectively (P=0.199). Additionally, log-rank analysis of Kaplan-Meier curves showing case volume did not reveal a significant difference in long-term survival between the HV and LV centers (P=0.272), though the LV centers had wide differences for long-term outcomes (P=0.030). Case volume did not have effects on short- or long-term outcomes following LT in Japan, while there were large variations in long-term outcomes among the LV centers compared to those of the HV centers.

Living-donor lobar lung transplantation

Living-donor lobar lung transplantation (LDLLT) is indicated for critically ill patients who would not survive the waiting period in the case of severe brain-dead donor shortage. It is essential to confirm that potential donors are willing to donate without applying psychological pressure from others. In standard LDLLT, the right and left lower lobes donated by 2 healthy donors are implanted into the recipient under cardiopulmonary support. LDLLT can be applied to various lung diseases including restrictive, obstructive, infectious, and vascular lung diseases in both adult and pediatric patients if size matching is acceptable. Functional size matching by measuring donor pulmonary function and anatomical size matching by 3-dimensional computed tomography volumetry are very useful. When 2 donors with ideal size matching are not available, various transplant procedures, such as single lobe, segmental, recipient lobe-sparing, and inverted lobar transplants are valuable options. There seems to be immunological advantages in LDLLT as compared to cadaveric lung transplantation (CLT). Unilateral chronic allograft dysfunction is a unique manifestation after bilateral LDLLT, which may contribute to better prognosis. The growth of adult lung graft implanted into growing pediatric recipients is suggested by radiologic evaluation. Although only 2 lobes are implanted, postoperative pulmonary function is equivalent between LDLLT and CLT. The long-term outcome after LDLLT is similar to or better than that after CLT. The author has performed 164 LDLLTs resulting in 71.6% survival rate at 10years. All living-donors returned to their previous life styles. Because of possible serious morbidity in donors, LDLLT should be applied only for critically ill patients.

Long-term management and outcome of lung transplantation in Japan.

The long-term survival after lung transplantation (LT) is favorable in Japan. However, long-term survivors after LT are subject to late complications, including chronic lung allograft dysfunction (CLAD), malignancy, infection, and chronic kidney disease (CKD) because of the need for lifelong immunosuppression. The rates of single cadaveric LT (CLT) and living-donor lobar LT (LDLLT) are higher than that of bilateral CLT in Japan. Here, we will describe the management of late complications and long-term outcome after LT in Japan. Attention should be paid to not only the phenotype of CLAD but also the difference in CLAD after CLT and after LDLLT as well as the timing of lung re-transplantation for advanced CLAD, especially after single CLT. Since post-transplant lymphoproliferative disorder is the most common malignancy after LT, infection monitoring for infection-related malignancies and appropriate screening are keys to the early diagnosis and treatment of malignancy after LT. The long-term management of infection after LT is also important, especially with regard to community-acquired pathogens, Aspergillus, and cytomegalovirus. When providing long-term care after LT, physicians should be aware of CKD and the timing of renal replacement therapy in cases with severe CKD. The widespread use of computed tomography and dialysis in Japan are beneficial for long-term survivors of LT. The similar survival outcomes of single CLT and LDLLT, compared with bilateral CLT, might contribute to improved long-term survival in Japan. Pulmonologists are encouraged to become further involved in long-term management after LT in Japan.

Bilateral lung transplant with pulmonary artery reconstruction using donor aorta for pulmonary hypertension with a giant pulmonary arterial aneurysm.

Standard bilateral lung transplantation (BLT) is not feasible for patients with pulmonary arterial hypertension (PAH) complicated with a giant pulmonary arterial aneurysm (PAA). This study aimed to describe the outcomes of BLT with pulmonary artery reconstruction (PAR) using donor aorta for such patients. This is a retrospective single-centre study reviewing PAH patients with a PAA who received BLT with PAR using donor aorta from January 2010 through December 2020. We compared the characteristics and short- and long-term outcomes of recipients receiving PAR (PAR group) with those who had no PAA and received standard BLT (non-PAR group). Nineteen adult PAH patients underwent cadaveric lung transplantation during the study period. Among them, 5 patients with a giant PAA (median pulmonary artery trunk diameter, 69.9 mm) underwent BLT with PAR using donor aorta and the others received standard BLT. Although the operation time tended to be longer in the PAR group compared with the non-PAR group (1239 vs 958 mins, P = 0.087), 90-day mortality (PAR group: 0% vs non-PAR group: 14.3%, P > 0.99), and 5-year survival rate (PAR group: 100% vs non-PAR group: 85.7%, P = 0.74) was comparable between the groups. No dilatation, constriction or infection of the aortic grafts were recorded during the study period with a median follow-up time of 94 months in the PAR group. Lung transplantation with PAR using donor aorta is a valid surgical option for PAH patients complicated with a giant PAA.

Diagnostic value of circulating microRNA-21 in chronic lung allograft dysfunction after bilateral cadaveric and living-donor lobar lung transplantation

BackgroundMicroRNAs (miRNAs) involved in the pathogenesis of pulmonary fibrosis have been shown to be associated with the development of chronic lung allograft dysfunction (CLAD) after lung transplantation (LT). We investigated the role of circulating miRNAs in the diagnosis of CLAD after bilateral LT, including cadaveric LT (CLT) and living-donor lobar LT (LDLLT). MethodsThe subjects of this retrospective study were 37 recipients of bilateral CLT (n = 23) and LDLLT (n = 14), and they were divided into a non-CLAD group (n = 24) and a CLAD group (n = 13). The plasma miRNA levels of the two groups were compared, and correlations between their miRNAs levels and percent baseline forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), and total lung capacity (TLC) values were calculated from one year before to one year after the diagnosis of CLAD. ResultsThe plasma levels of both miR-21 and miR-155 at the time of the diagnosis of CLAD were significantly higher in the CLAD group than in the non-CLAD group (miR-21, P = 0.0013; miR-155, P = 0.042). The miR-21 levels were significantly correlated with the percent baseline FEV1, FVC, and TLC value of one year before and at the time of diagnosis of CLAD (P < 0.05). A receiver operating characteristic curve analysis of the performance of miR-21 levels in the diagnosis of CLAD yielded an area under the curve of 0.89. ConclusionCirculating miR-21 appears to be of potential value in diagnosing CLAD after bilateral LT.

Comparison of living-donor lobar lung transplantation and cadaveric lung transplantation for pulmonary hypertension.

Living-donor lobar lung transplantation (LDLLT) is a life-saving procedure for critically ill patients with various lung diseases, including pulmonary hypertension (PH). However, there are concerns regarding the development of heart failure with pulmonary oedema after LDLLT in which only 1 or 2 lobes are implanted. This study aimed to compare the preoperative conditions and postoperative outcomes of LDLLT with those of cadaveric lung transplantation (CLT) in PH patients. Between 2008 and 2021, 34 lung transplants for PH, including 12 LDLLTs (5 single and 7 bilateral) and 22 bilateral CLTs, were performed. Preoperative variables and postoperative outcomes were retrospectively compared between the 2 procedures. Based on the preoperative variables of less ambulatory ability (41.7% vs 100%, P < 0.001), a higher proportion of World Health Organization class 4 (83.3% vs 18.2%, P < 0.001) and higher mean pulmonary artery pressure (74.4 vs 57.3 mmHg, P = 0.040), LDLLT patients were more debilitated than CLT patients. Nevertheless, hospital death was similar between the 2 groups (8.3% vs 9.1%, P > 0.99, respectively). Furthermore, the 5-year overall survival rate was similar between the 2 groups (90.0% vs 76.3%, P = 0.489). Although LDLLT patients with PH had worse preoperative conditions and received smaller grafts than CLT patients, LDLLT patients demonstrated similar perioperative outcomes and prognoses as CLT patients. LDLLT is a viable treatment option for patients with PH.

Living Donor Lobar Lung Transplant for a Patient With Lung Disease Caused by ABCA3 Gene Mutations: A Case Report

Recessive gene mutations in ABCA3 cause lethal neonatal respiratory distress, and pediatric and adult interstitial lung disease. The effectiveness of medical treatments is limited and a subset of such patients will eventually require lung transplantation. A 20 months old boy developed interstitial lung disease and was treated with hydroxychloroquine, which had a significant effect. Sequence analysis of ABCA3 gene revealed newly discovered compound heterozygous mutations. His respiratory dysfunction gradually progressed over years and he underwent living-donor lobar lung transplantation (LDLLT) at 8 years of age with his parents serving as bilateral lobar donors. The parents had been genetically examined beforehand and found to be carriers who had one allele with an ABCA3 gene mutation and the other with no mutation. The recipient has been well without chronic lung allograft dysfunction and his parents have been enjoying healthy social lives for 7 years since the operations. LDLLT appears to be a valid option for selected children with ABCA3 gene mutations who are too ill to wait for cadaveric lung transplantation. When relatives of the recipient with ABCA3 gene mutation are deemed potential donors for LDLLT, sequence analyses of the donors are indispensable to exclude the possibility that they are late-onset patients of this recessive hereditary disease.

Assessment of listing criteria for lung transplant candidates with interstitial lung disease.

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. 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. 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 22.5months. The 2-year survival rate was 47.8%. On multivariate Cox proportional hazards analysis, relative decline of percent predicted forced vital capacity (%FVC) in 6months ≥ 10% (hazard ratio [HR]: 1.72; 95% confidence interval [95%CI]: 1.03-2.87, p = 0.04) and 6-min walking distance (6MWD) < 250m (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). 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 6min walk distance may be useful for selecting patients with higher risks of mortality.

Thoracic mediastinal-occupying ratio predicts recovery and prognosis after lung transplantation.

OBJECTIVESEven 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.METHODSWe 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.RESULTSThe 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).CONCLUSIONSRecipients with a TMOR >40% may be long-term ventilator dependent and have a poor prognosis.

Long-term outcomes of living-donor lobar lung transplantation

ObjectiveAlthough living-donor lobar lung transplantation (LDLLT) enables an intermediate survival similar to cadaveric lung transplantation, the long-term outcome remains unknown. We examined the long-term outcomes of 30 patients who received LDLLT more than 16 years previously. MethodsWe retrospectively reviewed the clinical data of 30 patients who underwent LDLLT (bilateral LDLLT, 29 patients; single LDLLT, 1 pediatric patient) between October 1998 and April 2004. ResultsLDLLT was performed for 25 female and 5 male patients ranging in age from 8 to 55 years. The diagnoses included pulmonary hypertension (n = 11), pulmonary fibrosis (n = 7), bronchiolitis obliterans (n = 5), and others (n = 7). At a median follow-up of 205 months, 22 patients were alive and 8 were dead. The causes of death were infection (n = 3), malignancy (n = 2), acute rejection (n = 2), and chronic lung allograft dysfunction (CLAD; n = 1). Unilateral CLAD occurred in 17 patients (56.7%), but only 1 of these patients subsequently developed bilateral CLAD. Two patients underwent bilateral cadaveric lung retransplantations. The 5-, 10-, and 15-year CLAD-free survival rates were 80.0%, 62.8%, and 44.3%, respectively. Malignancy occurred in 7 patients. Two of 5 patients with chronic kidney disease requiring hemodialysis underwent living-donor kidney transplantation. The 5-, 10-, and 15-year overall survival rates were 96.7%, 86.7%, and 73.3%, respectively. ConclusionsAlthough only 2 lobes are implanted, LDLLT provides encouraging long-term outcomes. In patients with unilateral CLAD, the functioning contralateral graft might contribute to a favorable long-term outcome.

Emphysematous changes and lower levels of plasma irisin are associated with bronchiolitis obliterans syndrome after bilateral living-donor lobar lung transplantation.

Decreased irisin levels may be associated with the development of emphysema. Similarly, emphysematous changes may develop in patients with chronic lung allograft dysfunction (CLAD) after living-donor lobar lung transplantation (LDLLT). We investigated the severity of emphysematous changes and the relationship between irisin levels and CLAD after bilateral LDLLT and cadaveric lung transplantation (CLT). The subjects of this retrospective study were 59 recipients of bilateral LDLLT (n = 31) or CLT (n = 28), divided into a non-CLAD group (n = 41), a LDLLT-CLAD group (n = 11), and a CLT-CLAD group (n = 7). We compared the severity of emphysematous changes, the skeletal muscle mass, and the plasma irisin levels among the groups. The emphysematous changes were significantly more severe in the LDLLT-CLAD and CLT-CLAD groups (p = 0.046 and 0.036), especially in patients with bronchiolitis obliterans syndrome (BOS), than in the non-CLAD group. Although the skeletal muscle mass was similar in all the groups, the plasma irisin levels were significantly lower in the LDLLT-CLAD group (p = 0.022), especially in the patients with BOS after LDLLT, than in the non-CLAD group. Emphysematous changes and lower levels of plasma irisin were associated with CLAD, especially in patients with BOS, after bilateral LDLLT.

Various techniques for anastomosis of pulmonary arteries with size mismatch during lung transplantation.

Various techniques for anastomosis of pulmonary arteries with size mismatch during lung transplantation.

Importance of the preoperative prognostic nutritional index score as a predictor of chronic lung allograft dysfunction after lung transplantation: a Japanese single-institution study.

Numerous indicators have been discussed as predictive markers for the incidence of chronic allograft dysfunction (CLAD) after lung transplantation (LTX). The aim of this study was to evaluate whether or not the preoperative prognostic nutrition index (PNI) correlated with the development of CLAD. This study is a single-center and retrospective cohort study. Forty-six patients underwent cadaveric lung transplantation between 2000 and 2016 at our institution. The primary endpoint of this study was the CLAD-free survival of the patients. CLAD was diagnosed in 11 patients (23%) during the follow-up period. Potential risk factors included recipient factors, donor factors, number of HLA mismatches, operation-related factors, and preoperative blood test results, including the preoperative PNI. The patients with a higher PNI showed a longer CLAD-free survival after LTX than those with lower values according to univariate and multivariate analyses (p = 0.01, 0.04, respectively). The 5-year CLAD-free survival rates in the higher-PNI patients and lower-PNI patients were 94% and 62%, respectively. We found that a lower preoperative PNI of the recipient was significantly associated with a higher incidence rate of CLAD. The preoperative PNI may, therefore, be useful as a predictor of the development of CLAD.

Initial Experience with Cadaveric Lobar Lung Transplantation in Turkey

Initial Experience with Cadaveric Lobar Lung Transplantation in Turkey

Comparison of de novo donor-specific antibodies between living and cadaveric lung transplantation

Despite growing interest in donor-specific antibodies (DSAs) and antibody-mediated rejection (AMR) in lung transplantation (LTx), no study evaluating the outcomes in recipients with de novo DSAs (dnDSAs) in living-donor lobar LTx (LDLLT) exists. We compared various characteristics of DSAs in LDLLT with those in cadaveric LTx (CLT) based on prospectively collected data. Between October 2009 and September 2019, 211 recipients underwent LTx (128 CLTs and 83 LDLLTs). We reviewed 108 CLTs and 74 LDLLTs to determine the characteristics and clinical impact of dnDSAs. Eighteen data-deficient cases, 7 cases with preformed DSAs, and 4 re-transplants were excluded. There were significant differences between CLT and LDLLT patients in age, primary disease, ischemic time, and number of human leukocyte antigen mismatches per donor. The dnDSA incidence in LDLLT (6.8%) was significantly lower than that in CLT (19.4%, p = 0.02). The dnDSAs appeared later in LDLLT (mean 1,256 days) than in CLT (mean 196 days, p = 0.003). According to Cox models analyzed using dnDSA as a time-dependent covariate, dnDSA positivity was significantly associated with a poor overall survival (OS; hazard ratio [HR] 3.46, 95% confidence interval [CI] 1.59-7.57, p = 0.002) and poor CLAD-free survival in case of CLT (HR: 2.23, 95% CI: 1.08-4.63, p = 0.003). However, no such significant associations were noted in case of LDLLT. The dnDSA occurrence was significantly lower and later in LDLLT than in CLT. Furthermore, dnDSA-positivity was significantly associated with worse OS and CLAD-free survival after CLT but not after LDLLT.

Lung Transplantation with Pulmonary Artery Reconstruction Using Donor Aorta for Pulmonary Hypertension with Giant Pulmonary Arterial Aneurysm: Intermediate-Term Result

<h3>Purpose</h3> Giant pulmonary arterial aneurysm (PAA) is a rare but serious complication for patients with pulmonary hypertension (PH). Heart-lung transplantation or lung transplantation with a full length of donor pulmonary artery (PA) for pulmonary artery reconstruction (PAR) are options for PH complicated with a giant PAA involving PA trunk to main PAs. We have been using donor aorta for the reconstruction of PA for these lung transplant cases. This study aimed to describe our intermediate-term outcomes of lung transplantation with PAR using donor aorta for PH complicated with a giant PAA. <h3>Methods</h3> This is a single center retrospective review from March 2000 to May 2020. We reviewed cadaveric lung transplantations for PH and compared the outcomes of lung transplantation with PAR (PAR group) with those without PAR (non-PAR group). <h3>Results</h3> Twenty-five PH patients underwent cadaveric lung transplantation. Among them, 5 patients with a giant PAA underwent lung transplantation with PAR using donor aorta. Representative 3D-CT images of a giant PAA and the reconstructed PA are shown in Figure A. The diameter of the PA trunk was significantly larger in the PAR group compared to the non-PAR group (Figure B). As expected, operative time was significantly longer in the PAR group compared to the non-PAR group (PAR, 1204 minutes; non-PAR, 965 minutes, p=0.035). On the other hand, primary graft dysfunction grade, 30-day mortality (PAR, 0%; non-PAR, 5.0%), 5-year survival rate (PAR, 100%; non-PAR, 73.9%, Figure C), and 6 minutes walk distance at 1 year after lung transplantation (PAR, 522 m; non-PAR, 526m) were not significantly different between the groups. The donor aortas used for PAR did not show constriction, obstruction, enlargement, or infection during the observation period. <h3>Conclusion</h3> Our experience supports that lung transplantation with PAR using donor aorta is a feasible surgical option for PH complicated with a giant PAA.

Pediatric Lung Transplantation−Intermediate Outcomes of a Japanese Center

<h3>Purpose</h3> Since 1998 our center has performed not only cadaveric lung transplantation (CLTx) but living-donor lobar lung LTx (LDLLTx), especially recently, including non-standard procedures of middle lobe LTx and segmental LTx in order to save younger children. The aim of this study was to show outcomes of pediatric LTx of a lung transplant center in Japan. <h3>Methods</h3> We retrospectively reviewed 203 consecutive LTx recipients between 1998 and 2020 including 43 pediatric (< 18 years old: CLTx [n = 9], LDLLTx [n = 34]) and 160 adult (≥18 years old: CLTx [n = 99], LDLLTx [n = 60]) recipients. We analyzed pediatric recipients in subgroups divided at the school age (0-6 years old [n = 10] vs 7-17 years old [n = 33]). <h3>Results</h3> The 1-, 5-, and 10-year overall survival (OS) in pediatric vs adult recipients were 93% vs 91%, 84% vs 74% and, 79% vs 61%, respectively (p = 0.11). The 1-, 5-, and 10-year lung-graft survival (GS) in pediatric vs adult recipients were 85% vs 83%,62% vs 62% and 36% vs 48%, respectively (p = 0.26). There was no significant difference in survival rates between pediatric and adult recipients. In pediatric recipients, the 1- and 5-year OS in 0-6 years old vs 7-17 years old were 70% vs 100% and 56% vs 92%, respectively (p = 0.004). The 1- and 5-year GS in 0-6 years old vs 7-17 years old were 60% vs 93% and 24% vs 72%, respectively (p = 0.02). Although the number is small, younger children under 7 years old have poorer OS and GS when compared with older children of 7-17 years old. From patient characteristics, lower height (p < 0.0001), non-standard LTx procedure (p = 0.001),idiopathic interstitial pneumonia (p = 0.004) and on-ventilation at LTx (p = 0.007) might be factors associated with poor outcome. <h3>Conclusion</h3> Outcome of pediatric LTx is comparable with that of adult LTx. However, younger children under 7 years old have poorer OS and GS compared with older children of 7-17 years old.