Experience of Lung Transplantation in Patients with Lymphangioleiomyomatosis at a Brazilian Reference Centre.

INTRODUCTION: Lung transplantation (LT) is the standard of care for patients with advanced lung diseases, including lymphangioleiomyomatosis (LAM). LAM accounts for only 1% of all LTs performed in the international registry. As a result, the global experience, including the use of mechanistic target of rapamycin (mTOR) inhibitors before and after LT in LAM, is still limited. METHODS: We conducted a retrospective review of all LAM patients who underwent LT at our centre between 2003 and 2016. Pre- and post-transplant data were assessed. RESULTS: Eleven women with LAM underwent LT, representing 3.3% of all procedures. Ten (91%) patients underwent double-LT. The mean age at diagnosis was 39 ± 6 years and the mean FEV1 before LT was 28 ± 14%. Only one patient underwent pleurodesis for recurrent pneumothorax. Pulmonary hypertension was confirmed in 3 (27%) patients. Four (36%) patients received sirolimus preoperatively; three of them received it until the day of LT, and there was no occurrence of bronchial anastomotic dehiscence after the procedure. Four patients (36%) received mTOR inhibitors post-transplant. The median follow-up from LT was 44 months. There were 3 deaths (27%) during the study and survival probabilities at 1, 3, and 5 years after LT were, 90, 90, and 77%, respectively. CONCLUSIONS: This data reinforces the role of LT for LAM patients with end-stage disease. The use of sirolimus seems to be safe before LT and the occurrence of complications after LT, including those LAM-related, should be continuously monitored.

Brazilian initial experience with lung transplantation due to irreversible lung fibrosis post-COVID-19 in a national reference center: a cohort study.

BACKGROUND: Lung transplantation (LTx) has been discussed as an option for treating irreversible lung fibrosis post-coronavirus disease 2019 (COVID-19), in selected cases. OBJECTIVES: To report on the initial experience and management of end-stage lung disease due to COVID-19 at a national center reference in Brazil. DESIGN AND SETTING: Cohort study conducted at a national reference center for lung transplantation. METHODS: Medical charts were reviewed regarding patients' demographics and pre-COVID-19 characteristics, post-LTx due to COVID-19. RESULTS: Between March 2020 and September 2021, there were 33 cases of LTx. During this period, we evaluated 11 cases of severe COVID-19-related acute respiratory distress syndrome (ARDS) that were potentially candidates for LTx. Among these, LTx was only indicated for three patients (9.1%). All of these patients were on venovenous extracorporeal membrane oxygenation (ECMO), and the procedure that they underwent was central venoarterial ECMO. All three patients were still alive after the first 30 postoperative days. However, patient #1 and patient #2 subsequently died due to fungal sepsis on the 47th and 52nd postoperative days, respectively. Patient #3 was discharged on the 30th postoperative day. CONCLUSIONS: LTx is feasible among these complex patients. Survival over the first 30 days was 100%, and this favors surgical feasibility. Nonetheless, these were critically ill patients.

Extracorporeal Membrane Oxygenation and Lung Transplantation: Initial Experience at a Single Brazilian Center.

OBJECTIVE: To report initial experience from the use of extracorporeal membrane oxygenation (ECMO) in patients who received lung transplantation. METHODS: Retrospective study of a single tertiary center in the Brazilian state of São Paulo, a national reference in lung transplantation, based on the prospective collection of data from electronic medical records. The period analyzed extended from January 2009 (beginning of the program) until December 2018. RESULTS: A total of 75 lung transplants were performed, with ECMO used in 8 (10.7%) cases. Of the patients, 4 (50%) were female. The mean age was 46.4±14.3 years. The causes of the end-stage lung disease that led to transplantation were pulmonary arterial hypertension in 3 (37.5%) patients, bronchiectasis in 2 (25%) patients, pulmonary fibrosis in 2 (25%) patients, and pulmonary emphysema in 1 (12.5%) patient. In our series, 7 (87.5%) cases were sequential bilateral transplantations. Prioritization was necessary in 4 (50%) patients, and in 1 patient, ECMO was used as a bridge to transplantation. The ECMO route was central in 4 (50%), peripheral venovenous in 2 (25%) and peripheral venoarterial in 2 (25%) patients. The mean length of the intensive care unit (ICU) stay was 14±7.5 days and of the hospital stay was 34.1±34.2 days. The mean ECMO duration was 9.3±6.6 days with a 50% decannulation rate. Three patients were discharged (37.5%). CONCLUSION: Lung transplantation requires complex treatment, and ECMO has allowed extending the indications for transplantation and provided adjuvant support in the clinical management of these patients.

Assessment of Pulmonary Function before and after Sinus Surgery in Lung Transplant Recipients.

Introduction The association between sinus and lung diseases is well known. However, there are scarce studies regarding the effects of sinus surgery on pulmonary function in lung transplant recipients. The present study describes our experience with sinus surgery in lung transplant recipients with chronic rhinosinusitis. Objectives To assess the impact of sinus surgery for chronic rhinosinusitis on pulmonary function and on inpatient hospitalization days due to lower respiratory tract infection in lung transplant recipients. Methods A retrospective study conducted between 2006 and 2012 on a sample of lung transplant recipients undergoing sinus surgery for chronic rhinosinusitis. Pulmonary function, measured by forced vital capacity (FVC) and forced expiratory volume in the first second (FEV1), as well as inpatient hospitalization days due to lower respiratory tract infection, were compared 6 months before and 6 months after sinus surgery. Results The FEV1 values increased significantly, and the inpatient hospitalization days due to bronchopneumonia decreased significantly 6 months after sinus surgery. The preoperative and postoperative median FEV1 values were 2.35 and 2.68 respectively ( p = 0.0056 ). The median number of inpatient hospitalization days due to bronchopneumonia 6 months before and 6 months after surgery were 32.82 and 5.41 respectively ( p = 0.0013 ). Conclusion In this sample of lung transplant recipients with chronic rhinosinusitis, sinus surgery led to an improvement in pulmonary function and a decrease in inpatient hospitalization days due to bronchopneumonia.

Burkholderia cepacia, cystic fibrosis and outcomes following lung transplantation: experiences from a single center in Brazil.

OBJECTIVES: To evaluate the impact of Burkholderia cepacia complex colonization in cystic fibrosis patients undergoing lung transplantation. METHODS: We prospectively analyzed clinical data and respiratory tract samples (sputum and bronchoalveolar lavage) collected from suppurative lung disease patients between January 2008 and November 2013. We also subtyped different Burkholderia cepacia complex genotypes via DNA sequencing using primers against the recA gene in samples collected between January 2012 and November 2013. RESULTS: From 2008 to 2013, 34 lung transplants were performed on cystic fibrosis patients at our center. Burkholderia cepacia complex was detected in 13 of the 34 (38.2%) patients. Seven of the 13 (53%) strains were subjected to genotype analysis, from which three strains of B. metallica and four strains of B. cenocepacia were identified. The mortality rate was 1/13 (7.6%), and this death was not related to B. cepacia infection. CONCLUSION: The results of our study suggest that colonization by B. cepacia complex and even B. cenocepacia in patients with cystic fibrosis should not be considered an absolute contraindication to lung transplantation in Brazilian centers.

Brazilian initial experience with lung transplantation due to irreversible lung fibrosis post-COVID-19 in a national reference center: a cohort study

ABSTRACT BACKGROUND: Lung transplantation (LTx) has been discussed as an option for treating irreversible lung fibrosis post-coronavirus disease 2019 (COVID-19), in selected cases. OBJECTIVES: To report on the initial experience and management of end-stage lung disease due to COVID-19 at a national center reference in Brazil. DESIGN AND SETTING: Cohort study conducted at a national reference center for lung transplantation. METHODS: Medical charts were reviewed regarding patients' demographics and pre-COVID-19 characteristics, post-LTx due to COVID-19. RESULTS: Between March 2020 and September 2021, there were 33 cases of LTx. During this period, we evaluated 11 cases of severe COVID-19-related acute respiratory distress syndrome (ARDS) that were potentially candidates for LTx. Among these, LTx was only indicated for three patients (9.1%). All of these patients were on venovenous extracorporeal membrane oxygenation (ECMO), and the procedure that they underwent was central venoarterial ECMO. All three patients were still alive after the first 30 postoperative days. However, patient #1 and patient #2 subsequently died due to fungal sepsis on the 47th and 52nd postoperative days, respectively. Patient #3 was discharged on the 30th postoperative day. CONCLUSIONS: LTx is feasible among these complex patients. Survival over the first 30 days was 100%, and this favors surgical feasibility. Nonetheless, these were critically ill patients.

Psychological criteria for contraindication in lung transplant candidates: a five-year study.

Lung transplantation presents a wide range of challenges for multidisciplinary teams that manage the care of the recipients. Transplant teams should perform a thorough evaluation of transplant candidates, in order to ensure the best possible post-transplant outcomes. That is especially true for the psychologist, because psychological issues can arise at any point during the perioperative period. The objective of our study was to evaluate the psychological causes of contraindication to waiting list inclusion in a referral program for lung transplantation. We retrospectively analyzed data on psychological issues presented by lung transplant candidates, in order to understand these matters in our population and to reflect upon ways to improve the selection process.

Lung transplantation.

Lung transplantation is a globally accepted treatment for some advanced lung diseases, giving the recipients longer survival and better quality of life. Since the first transplant successfully performed in 1983, more than 40 thousand transplants have been performed worldwide. Of these, about seven hundred were in Brazil. However, survival of the transplant is less than desired, with a high mortality rate related to primary graft dysfunction, infection, and chronic graft dysfunction, particularly in the form of bronchiolitis obliterans syndrome. New technologies have been developed to improve the various stages of lung transplant. To increase the supply of lungs, ex vivo lung reconditioning has been used in some countries, including Brazil. For advanced life support in the perioperative period, extracorporeal membrane oxygenation and hemodynamic support equipment have been used as a bridge to transplant in critically ill patients on the waiting list, and to keep patients alive until resolution of the primary dysfunction after graft transplant. There are patients requiring lung transplant in Brazil who do not even come to the point of being referred to a transplant center because there are only seven such centers active in the country. It is urgent to create new centers capable of performing lung transplantation to provide patients with some advanced forms of lung disease a chance to live longer and with better quality of life.

Lung transplantation: overall approach regarding its major aspects.

Lung transplantation is a well-established treatment for patients with advanced lung disease. The evaluation of a candidate for transplantation is a complex task and involves a multidisciplinary team that follows the patient beyond the postoperative period. Currently, the mean time on the waiting list for lung transplantation in the state of São Paulo, Brazil, is approximately 18 months. For Brazil as a whole, data from the Brazilian Organ Transplant Association show that, in 2014, there were 67 lung transplants and 204 patients on the waiting list for lung transplantation. Lung transplantation is most often indicated in cases of COPD, cystic fibrosis, interstitial lung disease, non-cystic fibrosis bronchiectasis, and pulmonary hypertension. This comprehensive review aimed to address the major aspects of lung transplantation: indications, contraindications, evaluation of transplant candidates, evaluation of donor candidates, management of transplant recipients, and major complications. To that end, we based our research on the International Society for Heart and Lung Transplantation guidelines and on the protocols used by our Lung Transplant Group in the city of São Paulo, Brazil.

Extracorporeal membrane oxygenation and lung transplantation: initial experience at a single brazilian center

OBJECTIVE: To report initial experience from the use of extracorporeal membrane oxygenation (ECMO) in patients who received lung transplantation. METHODS: Retrospective study of a single tertiary center in the Brazilian state of São Paulo, a national reference in lung transplantation, based on the prospective collection of data from electronic medical records. The period analyzed extended from January 2009 (beginning of the program) until December 2018. RESULTS: A total of 75 lung transplants were performed, with ECMO used in 8 (10.7%) cases. Of the patients, 4 (50%) were female. The mean age was 46.4±14.3 years. The causes of the end-stage lung disease that led to transplantation were pulmonary arterial hypertension in 3 (37.5%) patients, bronchiectasis in 2 (25%) patients, pulmonary fibrosis in 2 (25%) patients, and pulmonary emphysema in 1 (12.5%) patient. In our series, 7 (87.5%) cases were sequential bilateral transplantations. Prioritization was necessary in 4 (50%) patients, and in 1 patient, ECMO was used as a bridge to transplantation. The ECMO route was central in 4 (50%), peripheral venovenous in 2 (25%) and peripheral venoarterial in 2 (25%) patients. The mean length of the intensive care unit (ICU) stay was 14±7.5 days and of the hospital stay was 34.1±34.2 days. The mean ECMO duration was 9.3±6.6 days with a 50% decannulation rate. Three patients were discharged (37.5%). CONCLUSION: Lung transplantation requires complex treatment, and ECMO has allowed extending the indications for transplantation and provided adjuvant support in the clinical management of these patients.

Burkholderia cepacia, cystic fibrosis and outcomes following lung transplantation: experiences from a single center in Brazil

OBJECTIVES: To evaluate the impact of Burkholderia cepacia complex colonization in cystic fibrosis patients undergoing lung transplantation. METHODS: We prospectively analyzed clinical data and respiratory tract samples (sputum and bronchoalveolar lavage) collected from suppurative lung disease patients between January 2008 and November 2013. We also subtyped different Burkholderia cepacia complex genotypes via DNA sequencing using primers against the recA gene in samples collected between January 2012 and November 2013. RESULTS: From 2008 to 2013, 34 lung transplants were performed on cystic fibrosis patients at our center. Burkholderia cepacia complex was detected in 13 of the 34 (38.2%) patients. Seven of the 13 (53%) strains were subjected to genotype analysis, from which three strains of B. metallica and four strains of B. cenocepacia were identified. The mortality rate was 1/13 (7.6%), and this death was not related to B. cepacia infection. CONCLUSION: The results of our study suggest that colonization by B. cepacia complex and even B. cenocepacia in patients with cystic fibrosis should not be considered an absolute contraindication to lung transplantation in Brazilian centers.

Assessment of Pulmonary Function before and after Sinus Surgery in Lung Transplant Recipients

Abstract Introduction The association between sinus and lung diseases is well known. However, there are scarce studies regarding the effects of sinus surgery on pulmonary function in lung transplant recipients. The present study describes our experience with sinus surgery in lung transplant recipients with chronic rhinosinusitis. Objectives To assess the impact of sinus surgery for chronic rhinosinusitis on pulmonary function and on inpatient hospitalization days due to lower respiratory tract infection in lung transplant recipients. Methods A retrospective study conducted between 2006 and 2012 on a sample of lung transplant recipients undergoing sinus surgery for chronic rhinosinusitis. Pulmonary function, measured by forced vital capacity (FVC) and forced expiratory volume in the first second (FEV1), as well as inpatient hospitalization days due to lower respiratory tract infection, were compared 6 months before and 6 months after sinus surgery. Results The FEV1 values increased significantly, and the inpatient hospitalization days due to bronchopneumonia decreased significantly 6 months after sinus surgery. The preoperative and postoperative median FEV1 values were 2.35 and 2.68 respectively (p = 0.0056). The median number of inpatient hospitalization days due to bronchopneumonia 6 months before and 6 months after surgery were 32.82 and 5.41 respectively (p = 0.0013). Conclusion In this sample of lung transplant recipients with chronic rhinosinusitis, sinus surgery led to an improvement in pulmonary function and a decrease in inpatient hospitalization days due to bronchopneumonia.

Lung transplantation / Transplante pulmonar

ABSTRACT Lung transplantation is a globally accepted treatment for some advanced lung diseases, giving the recipients longer survival and better quality of life. Since the first transplant successfully performed in 1983, more than 40 thousand transplants have been performed worldwide. Of these, about seven hundred were in Brazil. However, survival of the transplant is less than desired, with a high mortality rate related to primary graft dysfunction, infection, and chronic graft dysfunction, particularly in the form of bronchiolitis obliterans syndrome. New technologies have been developed to improve the various stages of lung transplant. To increase the supply of lungs, ex vivo lung reconditioning has been used in some countries, including Brazil. For advanced life support in the perioperative period, extracorporeal membrane oxygenation and hemodynamic support equipment have been used as a bridge to transplant in critically ill patients on the waiting list, and to keep patients alive until resolution of the primary dysfunction after graft transplant. There are patients requiring lung transplant in Brazil who do not even come to the point of being referred to a transplant center because there are only seven such centers active in the country. It is urgent to create new centers capable of performing lung transplantation to provide patients with some advanced forms of lung disease a chance to live longer and with better quality of life.

RESUMO O transplante pulmonar é um tratamento mundialmente aceito para alguma pneumopatias avançadas, conferindo aos receptores maior sobrevida e melhor qualidade de vida. Desde o primeiro transplante realizado com sucesso em 1983, mais de 40 mil transplantes foram feitos em todo mundo. Destes, cerca de 700 foram no Brasil. No entanto, a sobrevida do transplante é menor do que a desejada, com altos índices de mortalidade relacionados à disfunção primária do enxerto, infecções e disfunção crônica do enxerto, principalmente sob a forma da síndrome da bronquiolite obliterante. Novas tecnologias têm sido desenvolvidas para aprimoramento das diversas etapas do transplante pulmonar. Para aumentar a oferta de pulmões, o recondicionamento pulmonar ex vivo vem sendo utilizado em alguns países, inclusive no Brasil. Para suporte avançado de vida no período perioperatório, equipamentos de oxigenação extracorpórea e de suporte hemodinâmico vêm sendo utilizado como ponte para o transplante em pacientes gravemente doentes em lista de espera e para manter pacientes vivos até a resolução da disfunção primária do enxerto pós-transplante. Existe uma demanda reprimida de pacientes que necessitam de transplante pulmonar no Brasil e que nem sequer chegam a ser encaminhados a um centro transplantador, pois só existem sete deles ativos no país. É urgente a criação de novos centros capazes de realizar transplante pulmonar para oferecer a pacientes com algumas pneumopatias avançadas uma chance de viver mais e com melhor qualidade de vida.

Lung transplantation: overall approach regarding its major aspects / Transplante pulmonar: abordagem geral sobre seus principais aspectos

ABSTRACT Lung transplantation is a well-established treatment for patients with advanced lung disease. The evaluation of a candidate for transplantation is a complex task and involves a multidisciplinary team that follows the patient beyond the postoperative period. Currently, the mean time on the waiting list for lung transplantation in the state of São Paulo, Brazil, is approximately 18 months. For Brazil as a whole, data from the Brazilian Organ Transplant Association show that, in 2014, there were 67 lung transplants and 204 patients on the waiting list for lung transplantation. Lung transplantation is most often indicated in cases of COPD, cystic fibrosis, interstitial lung disease, non-cystic fibrosis bronchiectasis, and pulmonary hypertension. This comprehensive review aimed to address the major aspects of lung transplantation: indications, contraindications, evaluation of transplant candidates, evaluation of donor candidates, management of transplant recipients, and major complications. To that end, we based our research on the International Society for Heart and Lung Transplantation guidelines and on the protocols used by our Lung Transplant Group in the city of São Paulo, Brazil.

RESUMO O transplante pulmonar é uma terapia bem estabelecida para pacientes com doença pulmonar avançada.A avaliação do candidato para o transplante é uma tarefa complexa e envolve uma equipe multidisciplinar que acompanha o paciente para além do período pós-operatório.O tempo médio atual em lista de espera para transplante pulmonar é de aproximadamente 18 meses no estado de São Paulo. Em 2014, dados da Associação Brasileira de Transplante de Órgãos mostram que 67 transplantes pulmonares foram realizados no Brasil e que 204 pacientes estavam na lista de espera para transplante pulmonar.O transplante pulmonar é principalmente indicado no tratamento de DPOC, fibrose cística, doença intersticial pulmonar, bronquiectasia não fibrocística e hipertensão pulmonar.Esta revisão abrangente teve como objetivos abordar os aspectos principais relacionados ao transplante pulmonar: indicações, contraindicações, avaliação do candidato ao transplante, avaliação do candidato doador, gestão do paciente transplantado e complicações maiores. Para atingirmos tais objetivos, utilizamos como base as diretrizes da Sociedade Internacional de Transplante de Coração e Pulmão e nos protocolos de nosso Grupo de Transplante Pulmonar localizado na cidade de São Paulo.