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BACKGROUND: The shortage of donors for lung transplants is the main limitation of the preceding. Lobar transplantation is an alternative especially useful in patients with short stature and small thoracic cavities. The aim of this study was to perform a descriptive analysis of Portuguese patients who underwent lobar lung transplantation. METHODS: A retrospective study was conducted, and patients submitted to lobar lung transplantation from January 2012 to December 2023 were evaluated. A descriptive analysis was made, including demographic data, lung diseases, waiting list dynamics, pre-transplant evaluations, and post-transplant outcomes. RESULTS: Sixteen lobar transplants were performed with a predominance of female patients and a median age of 47 years. Most patients had interstitial lung disease or bronchiectasis either due to cystic fibrosis or non-cystic fibrosis. The median predicted total lung capacity (pTLC) ratio was 0.73. The median waiting list time was 6 months with 9 urgent transplants and 1 emergent lobar retransplant. Extracorporeal membrane oxygenation (ECMO) was used in pre-, intra-, and postoperative periods. Most transplanted lobes were the median lobe (ML) + right upper lobe (RUL) and left upper lobe (LUL). The median length of stay was 58 days, with complications such as PDG grade 3, bronchial tree ischemia, and concentrical stenosis of bronchial anastomosis. Six patients died in this period, 1 in the immediate postoperative period and 5 during the post-transplant hospitalization, with a median survival of 20.7 months and a 1-year and 5-year survival rate of 60%. CONCLUSION: Our results show a population with an increased waiting list converging in many urgent cases, with an early mortality and high primary graft dysfunction rate. Nevertheless, mid- and long-term survival are promising.
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Transplante de Pulmão , Listas de Espera , Humanos , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Masculino , Adulto , Portugal , Resultado do TratamentoRESUMO
BACKGROUND: In patients with pulmonary arterial hypertension (PAH), refractory to medical therapy, lung transplantation emerges as an option. This study describes the outcomes of 8 PAH patients who underwent lung transplantation. METHODS: A retrospective, single-center study was conducted among patients with PAH who underwent lung transplantation in our center. RESULTS: Patients had a median age of 46 years, with female sex predominance (75%). Causes of HAP were pulmonary veno-occlusive disease (n = 5, 62.5%), idiopathic PAH (n = 2, 25%), and heritable PAH (n = 1, 12.5%). Pre-transplant hemodynamics revealed a median mean pulmonary artery pressure of 58.5 mm Hg (48-86). All patients received bilateral lung transplants with extracorporeal membrane oxygenation support, displaying immediate post-transplant hemodynamic improvement. Primary graft dysfunction grade 3 (PGD 3) was observed in 75% of patients. Five patients (62.5%) died, with a 72.9% survival at 12 months and 29.2% at 24 months post-transplantation. CONCLUSION: Our study reveals the complexity and challenges of lung transplants in patients with PAH. Despite notable immediate hemodynamic improvements, high rates of PGD 3 and the survival rate remain a concern. Further research to define optimal peri and post-transplant management to improve survival is required.
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Transplante de Pulmão , Hipertensão Arterial Pulmonar , Humanos , Feminino , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto , Portugal , Hipertensão Arterial Pulmonar/cirurgia , Resultado do Tratamento , Oxigenação por Membrana Extracorpórea , Hemodinâmica , Disfunção Primária do Enxerto/etiologia , Hipertensão Pulmonar/cirurgia , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/mortalidadeRESUMO
Alpha 1-antitrypsin deficiency is an inherited autosomal codominant disorder, which predisposes patients to lung and/or liver disease. Even though it is considered rare, it is one of the most frequent genetic disorders worldwide, albeit remaining underdiagnosed. Several organizations and societies, including the Portuguese Society of Pulmonology have been elaborating guidelines and recommendations for the diagnosis and management of alpha 1-antitrypsin deficiency. Nevertheless, some important matters are yet to be included in those, mainly due to lack of robust scientific evidence, and continue to represent a point of discussion. This article reviews some important scientific publications and expresses the perspectives of a group of Portuguese experts regarding the management of alpha 1-antitrypsin deficiency, namely in terms of the pre and neonatal diagnosis, the impact of the COVID-19 pandemic, the validity of replacement therapy in lung transplant-receiving, and finally, alternative strategies of alpha 1-antitrypsin deficiency treatment to improve the patients' quality of life.
A deficiência de alfa 1-antitripsina é uma doença hereditária autossómica codominante que aumenta a predisposição para o desenvolvimento de doença pulmonar e/ou hepática. Esta doença, embora seja considerada rara, é um dos distúrbios genéticos mais comuns em todo o mundo. Contudo, atualmente ainda constitui uma doença subdiagnosticada. Várias organizações e sociedades, incluindo a Sociedade Portuguesa de Pneumologia, elaboraram recomendações e diretrizes para o diagnóstico e gestão da deficiência de alfa 1-antitripsina. Porém, estes documentos ainda não abordam alguns temas relevantes associados à gestão da deficiência de alfa 1-antitripsina, principalmente devido à falta de robustez na evidência científica, que continuam a representar um ponto de discussão entre a comunidade médica. Neste artigo é feita a revisão de publicações científicas relevantes acerca da deficiência de alfa 1-antitripsina, e são descritas as perspetivas de especialistas portugueses sobre a gestão da deficiência de alfa 1-antitripsina, nomeadamente ao nível do diagnóstico pré e neonatal, do impacto da pandemia COVID-19, da validação da terapêutica de aumento em doentes que receberam um transplante pulmonar e, por fim, estratégias alternativas para a melhoria do tratamento da deficiência de alfa 1-antitripsina de modo a promover a qualidade de vida dos doentes.
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COVID-19 , Deficiência de alfa 1-Antitripsina , Recém-Nascido , Humanos , Deficiência de alfa 1-Antitripsina/diagnóstico , Deficiência de alfa 1-Antitripsina/genética , Deficiência de alfa 1-Antitripsina/terapia , alfa 1-Antitripsina/genética , alfa 1-Antitripsina/uso terapêutico , Pandemias , Qualidade de VidaRESUMO
A significant dysfunction of another organ is usually considered an absolute contraindication for lung transplantation, unless multiorgan transplantation is indicated and practical, as is the case of combined lung-kidney transplantation. Few cases of combined lung-kidney transplantation have been described in the literature; however, it is known that, in certain cases, it is the only way to offer an opportunity to selected patients with renal and lung dysfunction. The authors are not aware of any previously published case of a patient receiving both extracorporeal membrane oxygenation and continuous venovenous hemodiafiltration as a bridge for combined kidney-lung transplantation. The authors present the first case of combined lung-kidney transplantation performed in Portugal.
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Thrombotic microangiopathy (TMA) is a pathologic condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ injury due to microvascular endothelial lesions and thrombosis. It occurs in a variety of diseases and, unless recognized and treated, leads to severe morbidity and mortality. We present the case of a 48-year-old woman who underwent lung transplantation, initially under tacrolimus, mycophenolate mofetil (MMF), and prednisolone. Several complications emerged in the following months, including abdominal aortic and left renal artery thrombosis and cutaneous infections, although her renal function remained normal. Six months after transplant, her renal function began to deteriorate, which was assumed to be due to elevated tacrolimus levels and doses were adjusted. Due to leukopenia, MMF was changed to everolimus. One year after, she was admitted with fatigue, anemia, and renal dysfunction. Complementary exams revealed only iron deficiency, leukopenia, normal platelets, and elevated lactate dehydrogenase; her renal ultrasound was normal. A renal biopsy was performed and thrombotic microangiopathy was subsequently identified as the main cause of the renal dysfunction. Tacrolimus was therefore discontinued and MMF restarted with slow improvement of renal function. Only when everolimus was stopped did the patient's renal function show incremental improvement. TMA may be a serious complication after lung transplantation and the risk is higher when a combination of tacrolimus and everolimus is used. Renal biopsy findings are essential to confirm the final diagnosis of TMA, allowing for a change in immunosuppression to prevent permanent and severe renal damage.
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Hospedeiro Imunocomprometido , Terapia de Imunossupressão/efeitos adversos , Nefropatias/imunologia , Transplante de Pulmão , Microangiopatias Trombóticas/imunologia , Everolimo/uso terapêutico , Feminino , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Transplante de Pulmão/efeitos adversos , Pessoa de Meia-Idade , Ácido Micofenólico/uso terapêutico , Tacrolimo/efeitos adversosRESUMO
BACKGROUND: Severe exacerbations and mortality are major outcomes in COPD, and risk factors for these events are actively searched for. Several predictors of mortality have been identified in COPD. The inspiratory capacity/total lung capacity (IC/TLC) ratio has been shown to be a strong predictor of all cause and respiratory mortality in patients with COPD. The major objectives of this study were to analyze which clinical parameters, including lung volumes, were the best predictors of the 5-year cumulative risk of hospital admissions or death and the 5-year risk of exacerbations, in stable COPD patients. METHODS: This study retrospectively reviewed data from 98 stable COPD patients, consecutively recruited in 2012. Forced expiratory volume in 1 s (FEV1), modified Medical Research Council dyspnea scale, exacerbation history (ExH), Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2011 groups, and lung volumes were reviewed. Five years later, this population was evaluated for cumulative exacerbations, hospital admissions, and mortality. All the population, and GOLD group D separately, were analyzed. RESULTS: The cumulative 5-year combined risk of hospital admission or death was significantly predicted by the ExH and the IC/TLC ratio. Analyzing separately group D, FEV1 was the only predictor of this outcome. The frequency of exacerbations in the previous year was the best predictor of future cumulative 5-year risk of subsequent exacerbations, both for the total population and the GOLD D group. CONCLUSION: ExH and IC/TLC ratio were the best predictors of the most severe outcomes in COPD (admissions or mortality), independently of COPD severity. FEV1 was the only predictor of the cumulative 5-year combined risk of hospital admission or death in the GOLD D group. ExH was the best predictor of 5-year cumulative future risk of exacerbations. Besides FEV1 and ExH, the IC/TLC ratio can be a useful predictor of severe outcomes in COPD.
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Capacidade Inspiratória , Pulmão/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/mortalidade , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Capacidade Pulmonar Total , Idoso , Idoso de 80 Anos ou mais , Progressão da Doença , Feminino , Volume Expiratório Forçado , Humanos , Medidas de Volume Pulmonar , Masculino , Pessoa de Meia-Idade , Admissão do Paciente , Prognóstico , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Capacidade VitalRESUMO
INTRODUCTION: Several studies suggest that severe asthmatic patients under oral steroids show higher risk of myopathy induced by steroids. Nevertheless, no conclusive data exist about the prolonged oral steroid therapy effects on the res- piratory muscles function. We didn't found correlation between the severity of obs- truction (evaluated by FEV1), hyperinflation (residual volu- me- RV) and the muscle pressures in both groups. Also, there were no significant correlation between the G1 PImax and oral steroid accumulated dose. CONCLUSION: We found a significant compromise of the res- piratory muscle function in the both studied groups with severe asthma. The oral steroid therapy caused no additional adverse effect in muscle respiratory function even after a prolon- ged treatment. The compromise of the muscle respiratory function foun- ded can be justified by the prolonged exposition to inhaled steroids. Key-words: Asthma, respiratory muscles, oral steroids.