Lung transplantation in patients with Bronchiectasis : The experience of InCor/HCFMUSP

Abstract

AbstractIntroductionBronchiectasis is a disease characterized by the abnormal dilatation and deformation of one or more bronchia or bronchioles, caused by repeated infections and inflammations. It has many etiologies, and among the most important are post-infectious, idiopathic, cystic fibrosis, aspiration and gastro esophageal reflux disease and congenital immunodeficiency. The clinical presentation is variable, but in general goes with repeated infections of the lower airways, worsening of pulmonary function, pulmonary hypertension and respiratory failure, causing great impact in quality of life and associated with elevated morbidity and mortality.Bronchiectasis treatment is multimodal and it changes according to the patient's clinical presentation and condition, and it could be from the antibiotic therapy with anti-inflammatories in patients with few symptoms and in the initial stage of the disease, to surgical resection in patients with focal disease, in patients who do not answer to conventional treatment and in patients with non-controlled hemoptysis despite the action of interventionist radiology.Lung transplantation is the only effective treatment for patients with end stage disease. The transplantation has began as a treatment option for suppurative diseases with cystic fibrosis, and cystic fibrosis is today one of the most important lung transplant indications, whereas Bronchiectasis has a small percentage in the total of the lung transplant indications worldwide.In Brazil, however, the number of lung transplant indications for Bronchiectasis is similar to the number of indications for cystic fibrosis, and apparently patients with Bronchiectasis has similar characteristics to the patients with cystic fibrosis, as being young, with high mortality in waiting list but presenting better survival, and also substantial improvement in quality of life.The experience acquired by the group of Lung Transplant of the Instituto do Coração of the HCFMUSP, having accomplished 27 bilateral lung transplantations in a period of 7 years is the biggest Brazilian experience. So, this study aims to analyze the cohort study of the patients who underwent bilateral lung transplantation due to Bronchiectasis at InCor, between 2003 and 2010.ObjectivesThe main objectives of this study are:*To relate the experience of InCor with the treatment of patients who have Bronchiectasis in advanced stage, who underwent a bilateral lung transplantation;*To define risk factors to the development of complications and mortality of the lung transplant for patients who have Bronchiectasis;*To compare the survival curve of patients who underwent lung transplantation by suppurative diseases (Bronchiectasis and cystic fibrosis) with the survival curve of other diseases.Material and methodsThis will be a retrospective study analyzing data referring to hospital records of patients with Bronchiectasis who underwent bilateral lung transplantation in the InCor HCFMUSP in the period between January of 2003 and December of 2010, with post-operatory following up until December of 2011.The data of the receptor and the donor and data referring to the transplant and to the post-operatory will be analyzed by univariate and multivariate analysis and will be correlated to the variables of the ending, searching risk factors to the development of severe Primary Dysfunction of the Graft, acute rejection in the first year, hospital and surgical mortality, bronchial complications and incidence of BOS (Bronchiolitis Obliterans Syndrome). The survival curve of this sample will be done by the Kaplan-Meier curve and compared to the survival curve of the patients who underwent lung transplantation in the same period.