Lung transplantation for non-cystic fibrosis bronchiectasis in Turkey: An initial institutional experience

Background/objectiveLung transplantation is a well-established treatment in patients who have bronchiectasis with diffuse involvement, and with a progressive decline in respiratory function despite maximal medical therapy. We have aimed to present pre-transplantation factors and our results of lung transplantation for non-cystic fibrosis bronchiectasis.MethodsPatients who underwent lung transplantation for non-cystic fibrosis bronchiectasis between the dates of December 2016 and July 2019 were included. The patients' clinical parameters, pulmonary function tests, microbiological results, cardiac parameters, intraoperative data, and lung transplant outcomes were assessed retrospectively.ResultsBilateral lung transplantation for bronchiectasis were performed in eleven patients. The mean age was 36.5 years (range 22–57 years). There were 4 (36.4%) female patients and 7 (63.6%) male patients. All patients had a high score as per the bronchiectasis severity index (BSI). The FACED score was moderate in six patients and severe in five patients. Preoperative colonization with Pseudomonas aeruginosa was observed in five patients. Hospital mortality was 18.2% (2/11). The 1-year mortality was 27.2% (3/11). Eight patients were alive. The mean follow-up period of patients with survival was 28.2 months (range 13–42 months). One patient was diagnosed with chronic lung allograft dysfunction (CLAD). The 3-year survival rates were 73%.ConclusionLung transplantation for bronchiectasis with end-stage lung disease can improve the quality of life and increase survival in selected patients. Further studies are needed to identify the optimal time for lung transplantation referral due to the availability of limited data.     

Urgency-Code Lung Transplantation for Cystic Fibrosis: Experience and Results

Lung transplantation (LT) under urgency-code mechanical ventilation (UCMV) has been identified in the International Society for Heart and Lung Transplantation (ISHLT) Registry as a negative prognostic factor increasing the likelihood of mortality. The objective of this study was to review our experience of UCLT for with cystic fibrosis (CF) patients compared with elective LT (ELT). From October 1993 to October 2007, we performed 259 consecutive LTs in 250 patients, of whom 78 (31.20%) had CF. Our study group comprised CF patients who received UCLT (n = 23). The type of LT in the UCLT group was as follows: bipulmonary (18), left unipulmonary (2), and bilobar transplantation from cadavers (3). The UCLT group more often required cardiopulmonary bypass (CB) (P = .025), pulmonary tailoring (P = .030), and longer periods of pulmonary ischemia (P = .066) than the ELT group. We noticed a greater number of cases of pneumonia during the first postoperative month in the UCLT group. However, incidence of surgical complications, early and perioperative mortality, and episodes of acute and chronic rejection (bronchiolitis obliterans syndrome) did not differ between the groups. Survival rates at 1, 3, 5, and 10 years were 73.66%, 63.74%, 42.49%, and 42.49%, respectively, in the UCLT group (mean, 1927 [SE = 366] days) and 75.95%, 71.32%, 63.37%, and 63.37% in the ELT group (mean, 2946 [SE = 281] days; P = .3417). In our experience, UCLT in patients with CF is fully justified. Careful selection of such cases permits acceptable long-term survival rates to be achieved with no increase in early or perioperative mortality.     

Results of Lung Transplantation in Patients With Cystic Fibrosis

Lung transplantation (LT) is the only available option for patients with cystic fibrosis (CF) with end-stage lung disease. We reviewed our experience with LT in patients with end-stage CF (CFLT) to identify variables associated with survival and to compare the results with other indications for LT (OILT). Between October 1993 and October 2007, we performed 259 consecutive LTs in 250 patients for treatment of various end-stage pulmonary conditions. The indications for LT were CF in 78 patients idiopathic pulmonary fibrosis in 76, COPD in 64, bronchiectasis in 11, alfa-1-antitrypsin deficit in 5, primary pulmonary hypertension in 4, bronchiolitis obliterans syndrome in 4, and other indications in 11. Our study group comprised 78 patients with CF (30.11%) (CFLT). We observed significant differences in the actuarial survival between the CFLT and OILT groups. Perioperative mortality and the incidence of bronchiolitis obliterans syndrome were comparable in both groups. We found that in patients with CF, LT performed under urgency code (mechanical ventilation) showed no significant difference from LT performed electively insofar as long-term survival, early death, or perioperative death. The functional results in the CFLT group were excellent. We observed significant improvement in PaO₂, PaCO₂, forced vital capacity, and forced expiratory volume in the first second of expiration at 6, 12, and 36 months compared with the pretransplantation baseline values.     

Lung Transplantation in Cystic Fibrosis and the Impact of Extracorporeal Circulation

Introduction

Lung disease is the major cause of death among cystic fibrosis (CF) patients, affecting 80% of the population. The impact of extracorporeal circulation (ECC) during transplantation has not been fully clarified. This study aimed to evaluate the outcomes of lung transplantation for CF in a single center, and to assess the impact of ECC on survival.

Cardiopulmonary transplantation

The Registry of the International Society for Heart and Lung Transplantation reported 108,034 adult recipients of deceased donor heart transplants between January 1992 and June 2018. The 37th annual adult lung transplant report submitted data to the ISHLT TTX Registry on 67,493 adult recipients of deceased donor transplants between January 1, 1992 and June 30, 2018. In recent years more than 5000 heart transplants and approximately 4000 lung transplants have been performed annually across 388 centres worldwide. Heart transplantation is a proven surgical option for selected patients with advanced heart failure refractory to surgical or medical management. Lung transplantation is the definitive treatment for end-stage lung disease in patients who have failed medical therapy. More than 90% of adult patients presenting for heart transplantation have dilated cardiomyopathy or ischaemic cardiomyopathy. Anaesthetic principles for heart transplantation comprise full monitoring, including transoesophageal echocardiography, cardiostable anaesthesia and cardiac support with assessment and treatment of pulmonary vascular hypertension. Median survival after cardiac transplantation is 11.9 years. Lung transplantation includes single-lung, double-lung, bilateral sequential single-lung, heart–lung and lobar transplantation. The most common indication, representing more than one-third of all transplant recipients, is chronic obstructive pulmonary disease. Donor criteria have become more liberal and lung transplants may involve cardiopulmonary bypass. Pre-bypass air trapping can compromise cardiac function. Postoperative ventilation management should be guided by pH, not P_aCO₂. Thoracic epidural analgesia provides optimal pain relief without respiratory depression. Five-year survival after lung transplantation is approximately 65%.     

A comparison of CF and non-CF school-age children undergoing lung transplantation

Cystic fibrosis (CF) is a genetic disorder resulting in a chloride channel (CFTR) defect characterized by multi-organ damage. The primary cause of morbidity and mortality is end-stage obstructive lung disease. Lung transplantation is a treatment option, but is complicated by the risks of acute rejection, Bronchiolitis Obliterans syndrome (BOS) (graft dysfunction), and serious infection. This study sought to assess survival free from three major complications, namely BOS, acute rejection, and serious infection and also to compare overall survival among school-age CF transplantation recipients to non-CF recipients. We limited consideration to school-age children because they comprise a unique cohort in terms of linear and graft growth, immunity, pharmacokinetics and infection exposure as compared with infants, adolescents and adults. The OPTN national database was searched for period between January 1997 and August 2006 for children between 6 and 10 years of age undergoing lung transplantation. Children with CF were compared with non-CF recipients with regard to occurrence of BOS, infection-related hospitalizations, and acute rejections. Kaplan–Meier analyses were used for statistical comparisons of the two cohorts. There were 50 CF patients and 37 non-CF patients available for analysis from the OPTN database. Up to 5 years post-transplant, there were no statistically significant differences between CF and non-CF patients in overall survival, and survival free from BOS, acute rejections, or serious infections defined as those requiring hospitalization. Despite having an underlying systemic disease based on defective CFTR, CF school-age children receiving a lung transplant do not demonstrate more major complications or lower survival than non-CF children.     

Increased risk of PTLD in lung transplant recipients with cystic fibrosis

Background

Post-transplant lymphoproliferative disease (PTLD) is an important cause of morbidity and mortality following lung transplantation. Recipients with cystic fibrosis (CF) may have an increased risk of PTLD although the literature is limited to single center cohorts. Our primary aim is to examine PTLD in an adult lung transplant population by utilizing the International Society for Heart and Lung Transplantation Registry.

Lung Transplantation in Pulmonary Fibrosis: Challenging Early Outcomes Counterbalanced by Surprisingly Good Outcomes Beyond 15 Years

Interstitial lung disease (ILD) has been reported to have a poor outcome following lung transplantation due to difficulties getting ill recipients to transplantation and challenging early postoperative outcomes. To assess long-term outcomes for this cohort, we performed a retrospective 18-year chart review of all ILD lung transplant recipients. ILD single (SLT) and bilateral sequential lung transplantations (BSLT) were compared with all other lung transplant patients and International Society for Heart and Lung Transplantation (ISHLT) Registry data over the same time period. Of 585 lung transplantations, 90 (15%) were ILD (53 SLT, 37 BSLT); 67 (74%) were idiopathic pulmonary fibrosis (IPF), 9 (10%) were sarcoidosis, 9 (10%) were lymphangioleiomyomatosis, and 5 (6%) had other indications. Mean age was 52 years (range, 34-69 years). Actuarial survival at 1, 5, 10, 15, and 18 years compared favorably to all other lung transplantations performed (77% vs 83%, 51% vs 50%, 42% vs 26%, 28% vs 17%, and 28% vs 8%, respectively). IPF actuarial survival at 1, 5, and 10 years appeared superior to ISHLT Registry data (76% vs 72%, 50% vs 44%, and 34% vs 20%, respectively). There was equivocal survival between SLT and BSLT at 1, 5, and 10 years (78% vs 68%, 49% vs 50%, and 29% vs 50%, respectively). Our ILD figures compared favorably to lung transplantation for other diseases and international standards, while survival from SLT was as successful as BSLT both in the short and the longer term. Consideration should be given to utilizing SLT to maximize the allocation of donor lungs and to decrease waiting list mortality associated with IPF.     

The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis

BackgroundThe prevalence of fungal disease in cystic fibrosis (CF) and non-CF bronchiectasis is increasing and the clinical spectrum is widening. Poor sensitivity and a lack of standard diagnostic criteria renders interpretation of culture results challenging. In order to develop effective management strategies, a more accurate and comprehensive understanding of the airways fungal microbiome is required. The study aimed to use DNA sequences from sputum to assess the load and diversity of fungi in adults with CF and non-CF bronchiectasis.MethodsNext generation sequencing of the ITS2 region was used to examine fungal community composition (n = 176) by disease and underlying clinical subgroups including allergic bronchopulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, non-tuberculous mycobacteria, and fungal bronchitis. Patients with no known active fungal disease were included as disease controls.ResultsITS2 sequencing greatly increased the detection of fungi from sputum. In patients with CF fungal diversity was lower, while burden was higher than those with non-CF bronchiectasis. The most common operational taxonomic unit (OTU) in patients with CF was Candida parapsilosis (20.4%), whereas in non-CF bronchiectasis sputum Candida albicans (21.8%) was most common. CF patients with overt fungal bronchitis were dominated by Aspergillus spp., Exophiala spp., Candida parapsilosis or Scedosporium spp.ConclusionThis study provides a framework to more accurately characterize the extended spectrum of fungal airways diseases in adult suppurative lung diseases.     

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines

ObjectiveProvide recommendations to the cystic fibrosis (CF) community to facilitate timely referral for lung transplantation for individuals with CF.MethodsThe CF Foundation organized a multidisciplinary committee to develop CF Lung Transplant Referral Consensus Guidelines. Three workgroups were formed: timing for transplant referral; modifiable barriers to transplant; and transition to transplant care. A focus group of lung transplant recipients with CF and spouses of CF recipients informed guideline development.ResultsThe committee formulated 21 recommendation statements based on literature review, committee member practices, focus group insights, and in response to public comment. Critical approaches to optimizing access to lung transplant include early discussion of this treatment option, assessment for modifiable barriers to transplant, and open communication between the CF and lung transplant centers.ConclusionsThese guidelines will help CF providers counsel their patients and may reduce the number of individuals with CF who die without consideration for lung transplant.     

Pseudomonas aeruginosa infection,but not mono or dual-combination CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis

BackgroundChronic infection and an exaggerated inflammatory response are key drivers of the pathogenesis of cystic fibrosis (CF), especially CF lung disease. An imbalance of pro- and anti-inflammatory mediators, including dysregulated Th2/Th17 cells and impairment of regulatory T cells (Tregs), maintain CF inflammation. CF transmembrane conductance regulator (CFTR) modulator therapy might influence these immune cell abnormalities.MethodsPeripheral blood mononuclear cells and serum samples were collected from 108 patients with CF (PWCF) and 40 patients with non-CF bronchiectasis. Samples were analysed for peripheral blood lymphocytes subsets (Tregs; Th1-, Th1/17-, Th17- and Th2-effector cells) and systemic T helper cell-associated cytokines (interleukin [IL]-5, IL-13, IL-2, IL-6, IL-9, IL-10, IL-17A, IL-17F, IL-4, IL-22, interferon-γ, tumour necrosis factor-α) using flow cytometry.Results51% of PWCF received CFTR modulators (ivacaftor, ivacaftor/ lumacaftor or tezacaftor/ ivacaftor). There were no differences in proportions of analysed T cell subsets or cytokines between PWCF who were versus were not receiving CFTR modulators. Additional analysis revealed lower percentages of Tregs in PWCF and chronic pulmonary Pseudomonas aeruginosa infection; this difference was also present in PWCF treated with CFTR modulators. Patients with non-CF bronchiectasis tended to have higher percentages of Th2- and Th17-cells and higher levels of peripheral cytokines versus PWCF.ConclusionsChronic P. aeruginosa lung infection appears to impair Tregs in PWCF (independent of CFTR modulator therapy) but not those with non-CF bronchiectasis. Moreover, our data showed no statistically significant differences in major subsets of peripheral lymphocytes and cytokines among PWCF who were versus were not receiving CFTR modulators.     

Cardiopulmonary transplantation

The Registry of the International Society for Heart and Lung Transplantation reported 4196 heart transplants and 3812 lung transplants worldwide in 2015. The 100,000th heart transplant mark has been passed. Heart transplantation is a proven surgical option for selected patients who have advanced heart failure refractory to surgical or medical management. Lung transplantation is the definitive treatment for end-stage lung disease for patients who have failed medical therapy. More than 90% of adult patients presenting for heart transplantation have dilated cardiomyopathy or ischaemic cardiomyopathy. Anaesthetic principles for heart transplantation include full monitoring including transoesophageal echocardiography, cardiostable anaesthesia and cardiac support, and assessment and treatment of pulmonary vascular hypertension. Median survival after cardiac transplantation is 11.9 years. Lung transplantation includes single-lung, double-lung, bilateral sequential single-lung, heart-lung and lobar transplantation. The most common indication is chronic obstructive pulmonary disease, which represents more than one-third of all transplant recipients. Donor criteria are becoming more liberal. Lung transplants may involve cardiopulmonary bypass. Pre-bypass air trapping can compromise cardiac function. Postoperative ventilation management should be guided by pH, not PaCO₂. Thoracic epidural provides optimal analgesia without respiratory depression. Five year survival after lung transplantation is approximately 65%.     

A Case Report of Cystic Fibrosis Plus Tuberous Sclerosis: A Cautionary Tale Regarding Lung Transplantation

BackgroundCystic fibrosis (CF) and tuberous sclerosis complex (TSC) are 2 rare genetic diseases that often affect the lungs. Pulmonary compromise in TSC or CF can be severe enough to require lung transplantation. In rare instances patients with CF undergo pneumonectomy to control recurrent lung infections and lung necrosis affecting one lung more than the other. Lung transplantation in these patients is exceedingly rare because preexistent pneumonectomy increases the risk of lung transplant–associated morbidity and mortality.Case PresentationWe present the case of a young woman with co-occurrence of TSC and CF, who underwent left-sided pneumonectomy and, approximately 2 years later, right-sided single lung transplant. The posttransplant clinical course was complicated by phrenic nerve injury, ventilator dependency, Aspergillus endocarditis with embolic shower, and death. Pretransplant pneumonectomy, Aspergillus colonization, and posttransplant phrenic nerve injury contributed to the complex postoperative course, ventilatory dependence, and poor outcome.ConclusionThis cautionary case should alert physicians on the challenges associated with single lung transplant in patients with preexistent pneumonectomy.     

Outcomes of Lung Transplantation in Patients With Combined Pulmonary Fibrosis and Emphysema: A Single-Center Experience

BackgroundCombined pulmonary fibrosis and emphysema (CPFE) is a distinct clinical entity that can progress to end-stage lung disease. Patients with CPFE may develop pulmonary hypertension and face a predicted 1-year mortality of 60%. Lung transplantation is the only curative therapeutic option for CPFE. This report describes our experience after lung transplantation in patients with CPFE.MethodsThis retrospective, single-center study describes short- and long-term outcomes for adult patients who underwent lung transplant for CPFE.ResultsThe study included 19 patients with explant pathology-proven diagnosis of CPFE. The patients were transplanted between July 2005 and December 2018. Sixteen recipients (84%) had pulmonary hypertension before transplant. Of the 19 patients, 7 (37%) had primary graft dysfunction at 72 hours post-transplant. 1-, 3-, and 5-year freedom from bronchiolitis obliterans syndrome was 100%, 91% (95% CI, 75%–100%), and 82% (95% CI, 62%–100%), respectively. One-, 3-, and 5-year survival was 94% (95% CI, 84%–100%), 82% (95% CI, 65%–100%), and 74% (95% CI, 54%–100%), respectively.ConclusionOur experience demonstrates the safety and feasibility of lung transplant for patients with CPFE. Significant morbidity and mortality without lung transplant coupled with favorable post-transplant outcomes merit prioritization of CPFE in the Lung Allocation Score algorithm for lung transplant candidacy.     

Lung transplantation for cystic fibrosis: current concepts and one center's experience.

BACKGROUND: Although new approaches to the treatment of patients with cystic fibrosis (CF) are significantly prolonging their lives, most patients will eventually develop respiratory failure due to progressive bronchiectasis caused by chronic lung infection and inflammation and die from to respiratory failure. We examined our center's (University of Wisconsin Hospital and Clinics) experience with lung transplantation for patients with CF and reviewed the literature to examine current and evolving approaches to transplantation for this indication. METHODS: We reviewed all published literature pertaining to lung transplantation for CF through 2006, and we reviewed all aspects of transplantation for patients with CF at our institution from 1994 to 2005. RESULTS: Major complications following lung transplantation include acute rejection, bacterial infection, and bronchiolitis obliterans. Five-year survival at UWHC (Kaplan-Meier) is 67%, and survival was not adversely affected by transplanting patients receiving mechanical ventilation. The major cause of death for transplant recipients was bronchiolitis obliterans syndrome (BOS). CONCLUSIONS: Lung transplantation for CF is associated with acceptable survival rates and can improve quality of life. Lung transplant should be offered to all patients with advanced CF lung disease if they meet currently accepted inclusion and exclusion criteria.     

First 3 Years of the Hungarian Lung Transplantation Program

In this article we summarize the results of the first 3 years after launching the Hungarian Lung Transplantation Program.Patients and MethodsThe first lung transplant in Hungary was carried out on December 12, 2015, with the collaboration of the National Institute of Oncology and the Semmelweis University. Up to December 31, 2018, a total of 62 lung transplants were performed. Data were analyzed retrospectively. Patients were listed for lung transplant after the indication was established by the National Lung Transplantation Committee. Donor lungs were procured from brain-dead donors only.ResultsWithin this period our team was involved in 87 lung procurements, 61 of which resulted in bilateral lung transplant and 1 in single-sided transplant. The operative approach was unilateral thoracotomy (n = 1), bilateral thoracotomy (n = 1), or clamshell incision (n = 60) with venoarterial extracorporeal membrane oxygenation support. The underlying disease of the recipients was obstructive lung disease (n = 30), lung fibrosis (n = 11), cystic fibrosis (n = 18), primary pulmonary hypertension (n = 2), histiocytosis-X syndrome (n = 1), bronchiectasis (n = 2), lymphangioleiomyomatosis (n = 1), and retransplant because of bronchiolitis obliterans syndrome (n = 1). The youngest patient was 13 years of age, while the oldest was 65 years. Three patients died in the early postoperative phase. One-year survival was 80%.DiscussionThe number of cases rises steadily in the Hungarian Lung Transplantation Program, which is exceptional compared with the start of other centrums. The incidence of complications and mortality is comparable with those of other experienced centers around the world. Our future goal is to broaden our waiting list, thus increasing the number of lung transplants carried out.     

Results of Lung Transplantations Among Cystic Fibrosis Patients: A Single-Center Study

BackgroundLung transplantation remains the ultimate treatment for patients who have exhausted all other therapeutic options in the course of end-stage lung disease due to cystic fibrosis (CF). The aim of the study was to assess the results of lung transplantations performed via mini-thoracotomy in a single center.MethodsThis retrospective study assesses the survival and need for reoperation among 56 primary lung transplant recipients due to CF in a single center between 2018 and 2021. Intraoperative death was also assessed, yet it was established as an exclusion criterion for the post-transplant survival analysis.ResultsOnly one patient died intraoperatively (1.79%). Reoperation at an early postoperative stage was required among 2 patients (3.58%), due to vascular complication for one and pulmonary leakage for the other. Mortality at 30 days was 0%. In-hospital mortality was low (3.58%). Survival at 1, 2, and 3 years was respectively 87%, 85%, and 75%. Mean forced expiratory volume in 1 second as a percentage of predicted value at discharge was approximately 60% and did not decrease after 12 and 24 months. Mean BMI at 12-month follow-up was 20.11 (range, 13-28.7) with 71.4% of patients being qualified as presenting within the normal range of 18.5 to 24.9.Conclusions. Double lung transplantation is a safe and feasible surgical option. Despite being more technically difficult and challenging than clamshell approach for surgeons, it is more beneficial for patients.     

Study of a Cohort of Patients With Cystic Fibrosis and Isolation of Scedosporium spp.

IntroductionIn recent years an increase in the prevalence of colonization and infection by Scedosporium spp. in patients with cystic fibrosis (CF) has been observed. In this article, we study the frequency of isolation of Scedosporium spp. in an adult CF Unit, analyzing characteristics of the patients and predisposing factors.MethodsA retrospective observational study was conducted in 87 adult CF patients in whom the presence of positive culture for Scedosporium spp. was tested for a 5-year period (January 2012–July 2017). We recorded the following clinical variables: age, sex, body mass index, genotype, presence of pancreatic insufficiency, bacterial colonization, lung function, other complications, exacerbations and treatment, and the modified Bhalla score from the last high-resolution computed tomography. Results were analyzed with IBM SPSS Statistics Version 22.0 software.ResultsScedosporium spp. was isolated in 25.3% of patients. In the bivariate analysis, these patients showed a higher rate of Pseudomonas aeruginosa infection, worse score in the Bhalla classification (highlighting the following items: bronchiectasis, mucus plugs and bronchial generations), a slight decrease in the lung diffusion capacity and more frequently received inhaled antibiotics. In the logistic regression multivariate analysis, only the bronchial generations item was significant.ConclusionScedosporium spp. must be considered an emerging opportunistic pathogen in patients with CF whose clinical involvement, risk factors or need for treatment is unknown.     

Testicular Cancer in a Lung Transplant Patient With Cystic Fibrosis: A Case Report

BackgroundCystic fibrosis (CF) is one of the most common genetic disorders that develops from a mutation of the cystic fibrosis transmembrane regulator gene. Patients with CF are known to be at risk for malignancies, and lung transplantation–associated immunosuppression further increases this risk.Case ReportWe describe a case of a 29-year-old male patient with CF who developed testicular cancer 14 months after a lung transplantation. Immunosuppressive therapy included antithymocyte globulin induction and tacrolimus, mycophenolate, and prednisolone maintenance therapy as compared to standard alemtuzumab induction, followed by tacrolimus and prednisolone, as used in our center. He underwent semicastration and refused chemotherapy. Immunosuppressive treatment was changed to tacrolimus, everolimus, and prednisolone, which did not influence excellent graft function. This case report highlights the importance of uro-oncological observation of patients with CF following lung transplantations.     

Survival of Iranian patients on lung transplant waiting list: is there any difference?

Objective

Lung transplantation is the last treatment option for end-stage pulmonary diseases. Reviewing the characteristics of patients on the lung transplant waiting list is a helpful way to evaluate and prioritize the patients in need of special care. Because we have no information about mortality on the lung transplantation waiting list in Iran, the aim of this study was to evaluate the characteristics and survival rates of these patients.

Methods

The file of lung transplant candidates listed between 2005 and 2010 were evaluated for patient demographic data, type of disorder, waiting list time, and outcomes of death, transplantation, or alive.

Results

The 131 patients on our list in this period revealed a mean age of 37 ± 14 years with 86 (66%) males. The most common disorder among waiting list patients was pulmonary fibrosis (n = 52; 40%). Among the 17 (13%) patients who were transplanted, most (35%) suffered from pulmonary fibrosis. The mean waiting time to transplantation was 17.2 ± 11.8 months. Twenty-two patients (7%) died while on the waiting list. The mortality rate was unexpectedly highest among cystic fibrosis patients and then those with bronchiectasis. The mean survival time for all non-transplant patients based on the Kaplan-Meier method was 27.4 months and their 2-year survival rate was 74% based on life tables.

Conclusion

Although pulmonary fibrosis patients show the poorest survival on lung transplant waiting lists, in other countries, patients with cystic fibrosis and bronchiectasis displayed the worst survival on the Iranian list probably due to their poor rehabilitation and sputum evacuation. We concluded that it is necessary for every center to evaluate the characteristics of its patient cohort to match the activity according to the needs.