Thoracic transplantation
Lung Transplantation in Patients With Cystic Fibrosis

https://doi.org/10.1016/j.transproceed.2013.02.010Get rights and content

Abstract

Cystic fibrosis (CF) an autosomal recessive genetic disorder, affects many organs. The great majority of deaths occur due to respiratory failure after many years of chronic pulmonary infection. Despite recent progress in early detection by studies of genetic mutations and better understanding to treat nutritional and infectious states, lung transplantation is the CF treatment for most advanced cases. According to the International Society for Heart and Lung Transplantation (ISHLT) data, CF is the third most common reason for lung transplantation (16.8%) showing the best survival rate (60% at 5 years). We have described our experience in lung transplantation of CF patients between January 2000 and December 2011, reviewing medical charts of these patients were for gender, age, body mass index (BMI), comorbidities, disease duration, previous sputum gram stain, ischemic time, incidence of severe primary graft dysfunction (PGD Grade 3), intensive care unit (ICU) length of stay, and Kaplan-Meier survival. Among 150 lung transplantation, the 30 CF patients (20%) represented the second most common cause. The average age was 27.4 ± 9.2 years, with a slight predominance of males (n = 16; 53.3%). The average BMI was 18.9 ± 2.6. Most patients (60%) had pancreatic exocrine dysfunction. Also, 83.3% of patients showed a positive sputum culture for Pseudomonas, while Burkholderia cepacia was identified in only 4 patients (13.3%). The average time of the disease was 20.8 ± 9.7 years. All transplantation were bilateral with an average ischemic time of 472 ± 98.3 minutes and ICU length of stay of 9.9 ± 6.3 days. The survival rates at 1 and 5 years were 92% and 77%, respectively, corresponding to the best outcomes among underlying diseases, comparable with other worldwide series and better than the ISHLT reports. CF, the second most common cause for lung transplantation among our cases, showed the best survival rate among all causes. Our survival rate was comparable with other reports.

Section snippets

Methods

This retrospective cohort study examined medical charts and a prospective databank of patients who underwent transplantation between 2000 and 2011 with follow-up through September 2012. We assessed all patients diagnosed with CF including our adult and pediatric populations. The study was approved by our ethics committee. We collected recipient pretransplant variables: age, gender, body mass index (BMI), blood type, diabetes, pancreatic exocrine failure, duration of disease, secondary pulmonary

Results

Between 2000 and 2011, we performed 150 lung transplantations for chronic obstructive pulmonary disease (COPD) (26.7%), CF (20%), pulmonary fibrosis (20%), bronchiectasis (17.3%), lymphangioleiomyomatosis (6%), primary pulmonary hypertension (2.7%), or other causes (7.3%). There were 102 bilateral (68%), 21 right single (14%), and 27 left single (18%) procedures. The 30 patients who underwent lung transplantation for CF showed a mean age of 27.4 ± 9.2 years with 2 younger than 18 years. There

Discussion

Lung transplantation is a well-known treatment for CF end-stage lung disease accounting for 16.8% of all cases worldwide according to the ISHLT registry.1 The first successful heart-lung and lung transplantations were performed in 1983 and 1987, respectively.2 Although the first lung transplantation in our institution was performed in 1990, only after 2000 did it become a more common surgery routinely performed in our hospital. Since then we have performed 150 transplantations with a

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