Lung Transplantation for Cystic Fibrosis and Non-cystic Fibrosis Bronchiectasis: A Single-Center Experience

ObjectivesLung transplantation is a well-established treatment for selected patients with advanced cystic fibrosis (CF)- and non-cystic fibrosis (non-CF)--related bronchiectasis. Because the number of lung transplants performed for patients with non-CF bronchiectasis is much smaller than for patients with CF, little data is available regarding patient selection, choice of procedure, and outcomes.MethodsBetween November 1997 and December 2013, 42 patients with CF and 33 patients with non-CF bronchiectasis underwent lung transplantation at the Rabin Medical Center, Israel. We analyzed and compared pretransplant evaluation data and short- and long-term results in both groups.ResultsMedian survival for the CF group in our study was 8.4 years, compared with 7.1 years for the non-CF group (P = .098), similarly to that reported by the International Society for Heart and Lung Transplantation Registry data. The main survival difference between groups was in the early postoperative period. Both groups achieved similar peak forced expiratory volume in 1 second values and had stable lung function at the 3-year follow-up. Biopsy-proven rates of acute cellular rejection were low for both groups. Rates of chronic lung allograft dysfunction development did not differ between CF and non-CF recipients.ConclusionOur institutional experience confirms that lung transplantation is feasible for non-CF bronchiectasis, and results are comparable to our CF cohort. The increased early mortality in this study occurred from 1999 to 2008 and was probably related to surgical techniques used at the time. Overall, 3-year and 5-year survival were comparable with the International Society for Heart and Lung Transplantation Registry data. Non-CF bronchiectasis patients achieved and maintained satisfactory lung function.     

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.

Long-term non-invasive ventilation in cystic fibrosis -- experience over two decades

BackgroundNon-invasive ventilation (NIV) is accepted as a bridge to lung transplantation in cystic fibrosis (CF) but there is little evidence to support its use outside this setting.MethodsWe reviewed the records of all patients with CF who received domiciliary NIV at our centre between 1991 and 2010.ResultsOf 47 patients studied, 36% underwent lung transplantation, 28% died without transplantation and 30% remain alive on NIV. Median duration of NIV was 16 months (range 2–90). Mean FEV₁ fell by 212 ml over the year before NIV but increased by 18 ml in the following year (p < 0.01). Individual response to NIV was associated with lower baseline and more rapid decline in FEV₁. From 1991 to 2000, 70% underwent lung transplantation; from 2001 to 2010 only 27% were transplanted.ConclusionsNIV may slow or reverse the decline in lung function in advanced CF. NIV is increasingly used beyond a bridge to transplantation at our centre.     

A safe protocol for rapid desensitization in patients with cystic fibrosis and antibiotic hypersensitivity

BackgroundCF patients often demonstrate hypersensitivity to one or multiple antibiotics due to frequent and repeated exposures. Attempts at antibiotic desensitization in this population are historically complicated by higher reaction rates, failure to complete the procedure and consequent withholding of first-line therapy. This study evaluates the outcomes of a rapid desensitization protocol developed at our institution.MethodsWe retrospectively reviewed the medical records of 15 patients undergoing 52 rapid antibiotic desensitizations at Brigham and Women's Hospital and Children's Hospital Boston utilizing our protocol.ResultsMean FEV1 % predicted was 44.1 (SD 16.5), with two patients at <30% and one patient desensitized during bilateral lung transplantation. Adverse reactions during desensitization occurred in 13.4%, and most were mild. 100% of patients completed the protocol and ultimately tolerated subsequent full-strength antibiotic courses.ConclusionsCF patients with antibiotic hypersensitivity can safely receive first-line antibiotics via our rapid desensitization protocol, including those with severe obstructive lung disease.     

Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis

BackgroundElexacaftor-tezacaftor-ivacaftor induces rapid clinical improvement in patients with cystic fibrosis (CF) and advanced pulmonary disease, often leading to suspend the indication for lung transplantation. Yet no long-term data is available in lung transplant candidates.MethodsLung transplant candidates (defined as being waitlisted for lung transplantation or considered for listing within 3 months) who have initiated elexacaftor-tezacaftor-ivacaftor were identified in the French cohort of patients with CF and advanced pulmonary disease. Patients were prospectively followed to evaluate treatment safety and effectiveness from initiation to July 20th, 2021.ResultsAmong the 331 patients with advanced CF pulmonary disease who initiated elexacaftor-tezacaftor-ivacaftor, 65 were lung transplant candidates (17 listed for transplantation, 48 considered for listing within 3 months). Median [IQR] follow-up time was 363 [329; 377] days. At the end of the follow-up period, two patients were transplanted five and 11 days following treatment initiation, two were listed for transplantation, and 61 no longer met transplantation criteria. Improvement in percent predicted forced expiratory volume in 1 s (ppFEV₁) at one month was +13.4% (95% confidence interval, 10.3%-16.5%; P < 0.0001) and remained stable thereafter. Treatment burden decreased substantially, with an 86% decrease in the need for intravenous antibiotics, 59% for oxygen therapy and 62% for non-invasive ventilation.ConclusionIn lung transplant candidates eligible for elexacaftor-tezacaftor-ivacaftor, the rapid improvement following initiation of treatment persisted over one year with a reduction in treatment burden and lung transplantation could be safely deferred in most patients.     

Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia dolosa (genomovar VI)

Cystic fibrosis (CF) with severe lung disease is a well‐recognized indication for lung transplantation. Colonization with various organisms in CF patients may impact post‐transplant morbidity and mortality. Burkholderia cepacia complex (BCC) is made up of distinct genomovars with significant morbidity and mortality associated with B. cenocepacia (genomovar III) following lung transplant. The outcomes of patients infected with genomovar B. dolosa (genomovar VI) have yet to be described in the literature. We performed a retrospective chart review of all cystic fibrosis patients colonized with B. dolosa from our center who underwent lung transplantation (n = 11) at various medical centers across the US between 2000 and 2014. Survival rates were 73%, 53%, and 30% for 1, 3, and 5 years, respectively. Median survival was 44 months (95% CI = 11.1‐76.8). CF patients with B. dolosa that have undergone lung transplantation have decreased one‐year survival when compared to all patients transplanted with cystic fibrosis. Conditional 5‐year survival for B. dolosa‐infected patients was 43% in patients that survived the first year post‐transplant, suggesting that this first year is crucial in managing the infection. Importantly, the survival of the B. dolosa patients was higher than compared to previously reported survival rates of B. cenocepacia patients post‐transplant.     

Nasal polyposis in lung transplant recipients with cystic fibrosis

BackgroundChronic rhinosinusitis with nasal polyposis is common in patients with cystic fibrosis (CF). There are still many open questions regarding factors related to this condition. Furthermore, the prevalence of nasal polyposis and its implications for the outcomes in lung transplant recipients with cystic fibrosis are unknown.MethodsAll CF patients who underwent lung transplantation at our centre between November 1992 and December 2009 were included. Nasal polyp status was determined endoscopically at time sinus surgery and its relationships to gender, age at lung transplantation, Liou raw score, body mass index, FEV₁%predicted, diabetes mellitus, pre-transplant pseudomonas colonisation of the sinuses and the lungs, pre-transplant corticosteroid use and type of mutation of the CFTR gene were analysed. The post-transplant survival times and the incidence of bronchiolitis obliterans syndrome in patients with or without nasal polyposis were compared.ResultsNasal polyps were found in 19% (17 patients) of the 89 lung transplant recipients, whose data was available for statistical analysis. None of the factors analysed was related to the nasal polyp status. The post-transplant survival times and the incidence of bronchiolitis obliterans syndrome did not significantly differ between patients with or without nasal polyposis.ConclusionsCF-related nasal polyposis occurs in a relevant fraction of lung transplant recipients. A specific effect of nasal polyposis on post-transplant outcome could not be confirmed. Nevertheless, there was a trend to NP recurrence in patients with post‐transplant sinonasal pseudomonas colonisation and is a tendency of less chronic rejection in CF patients with nasal polyps.     

Diabetes mellitus and survival in cystic fibrosis patients after lung transplantation

BackgroundDiabetes mellitus (DM) is common in CF and associated with more severe disease. It is unclear whether DM influences outcome of lung transplantation (LTx).MethodsOne hundred twenty-three CF patients evaluated for LTx at our centre were included. Survival was calculated.ResultsPatients with and without DM did not differ with regard to gender, age, FEV₁ and BMI prior to LTx. Eighty patients (65%) had a diagnosis of DM before and 13 (11%) only after LTx. Recipients with DM had a significantly higher 1- and 5-year survival (89% and 71%) than those without (71% and 51%). In the multivariate Cox-regression analysis, DM had no impact on LTx-survival.ConclusionsOne- and five-year survival rates after LTx tend to be better in CF recipients with DM compared to those without DM. No impact of DM on the development of BOS was found.     

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.     

Liver transplantation in children with cystic fibrosis: a long-term longitudinal review of a single center's experience

Background: Improved long-term survival in cystic fibrosis (CF) has led to an increased incidence of extrapulmonary complications of this disease. Of these, end-stage liver disease is a significant cause of morbidity and mortality with liver transplantation being the only effective therapy.Methods: Records of all CF pediatric liver transplant recipients were reviewed.Results: Twelve children with CF were the recipients of 16 allografts. The 1- and 5-year survival was 91.6% and 75%, respectively. There were 5 deaths at a mean interval of 6.8 ± 6.3 years. All of these deaths were related to pulmonary disease. Pulmonary function improved or remained stable in 8 of 9 patients tested. Despite an 83% incidence of positive sputum cultures, there was only one early mortality related to pulmonary sepsis in the setting of primary liver allograft nonfunction.Conclusions: Liver transplantation is acceptable treatment for children with CF and end-stage liver disease. Long-term survival is comparable to liver transplantation performed for other indications. Although posttransplant morbidity and mortality is related to lung disease, the authors speculate that as therapeutic improvements prolong the survival in CF, it is expected that longer survival after liver transplantation in this patient population may also be anticipated.     

Various Aspects of Bacterial Infections in the Early Postoperative Stage Among Lung Transplant Recipients on Broad-Spectrum Antibiotics: A Single Center Study

BackgroundLife-long immunosuppression after lung transplantation increases the risk of bacterial infections, hence broad-spectrum antibiotics can be implemented after transplant. The aim of this study is to assess various aspects of bacterial infections in the early postoperative stage among lung transplant recipients on broad-spectrum antibiotics at a single center.MethodsThis retrospective study consists of 134 primary lung transplant recipients transplanted between 2014 and 2021 at a single center. Study analyzed the occurrence of de novo bacterium in bronchoalveolar lavage sampled 2 to3 weeks after lung transplantation, as well as survival and the occurrence of bacterial sepsis. Studied antibiotics include linezolid, meropenem, tobramycin, and cloxacillin.ResultsNone of the patients from the broad-spectrum antibiotics developed bacterial sepsis within the first 30 postoperative days. In-hospital mortality due to bacterial sepsis among patients in the broad-spectrum group was 1.89%. The most common new pathogen in first couple of days after lung transplantation was Burkholderia multivorans (42%). After its occurrence, Ceftazidime was administered. It significantly reduced the occurrence of hospital-acquired B multivorans after 2 to 3 weeks post-transplant (χ² = 8.01, P = .005).ConclusionBroad-spectrum antibiotics seem to be an efficient approach against bacterial infections for lung transplant recipients in the early post-transplant period, as patients treated this way very rarely develop fatal bacterial infections in the studied period. Ceftazidime proved efficient for treatment for B multivorans among the studied group. Patients, who acquired new pathogen during post-transplant hospital stay presented comparable lung function at discharge in comparison to those who were not.     

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.     

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.     

Survival in cystic fibrosis after acute respiratory failure supported by extracorporeal membrane oxygenation and/or invasive mechanical ventilation

BackgroundDespite therapeutic advances, people with cystic fibrosis (CF) develop progressive worsening and exacerbations of their lung disease, which can lead to acute respiratory failure. Historically, survival after mechanical ventilation (MV) has been poor. Outcomes related to use of extracorporeal membrane oxygenation (ECMO) have not been well described in CF.MethodsWe conducted a retrospective analysis of adult patients with CF admitted to the ICU for acute respiratory failure and requiring invasive MV with or without ECMO between July 1, 2006 and June 30, 2016. Separate analysis for the subgroup of MV patients who were eligible for transplant was conducted.ResultsMortality for all patients with respiratory failure requiring advanced support was 37%. Ten of 28 (36%) MV patients, 10 of 26 (38%) ECMO+MV patients and 7 of the 21 (33%) transplant eligible MV patients died. Intensive care unit (ICU) length of stay (LOS) was 24.5±16.6 days for ECMO+MV; 12.9±9.0 days for MV (p=0.001), and 12.3 ±10 days for transplant eligible MV patients (p=0.005 for ECMO+MV comparison). Seven transplant eligible MV patients (33%) and 16 ECMO+MV patients (62%) underwent lung transplantation (p<0.001) during the hospital admission. One and 2-year survival for individuals who survived ICU admission was similar regardless of mode of support. Cox-proportional hazards model did not yield any variables that significantly influenced ICU mortality, 1-year or 2-year mortality.ConclusionSurvival for CF patients with acute respiratory failure requiring MV with or without ECMO has improved over time. ECMO may be an appropriate modality for respiratory support in patients with CF and acute respiratory failure who have greater risk of death from MV alone.     

The effect of acute maximal exercise on the regional distribution of ventilation using ventilation MRI in CF

BackgroundThe importance of exercise in the management of people with CF is well recognised, yet the effect of exercise on lung function is not well understood. FEV₁ is insensitive to the detection of small changes in lung function. Ventilation MRI and LCI are both more sensitive to mild lung disease than FEV₁ and may be better suited to assess the effects of exercise. Here we assessed the short-term effects of maximal exercise on the distribution of ventilation using ventilation MRI and LCI.MethodsPatients with CF and a range of lung disease were assessed. Baseline LCI and ventilation MRI was followed by a maximal cardio-pulmonary exercise test (CPET). Repeated ventilation MRI was performed within 30 minutes of exercise termination, followed by LCI and finally by FEV₁.Results13 patients were recruited and completed all assessments. Mean (SD) age was 25 (10) years and mean (SD) FEV₁ z-score was -1.8 (1.7). Mean LCI at baseline was 8.2, mean ventilation defect percentage on MRI (VDP) was 7.3%. All patients performed maximal CPET. Post-exercise, there was a visible change in lung ventilation in 85% of patients, including two patients with increased ventilation heterogeneity post-CPET who had normal FEV₁. VDP and LCI were significantly reduced post-exercise (p < 0.05) and 45% of patients had a significant change in VDP.ConclusionsAcute maximal exercise directly affects the distribution of ventilation on ventilation MRI in patients with CF. This suggests that exercise is beneficial in CF and that ventilation MRI is suitable to assess airway clearance efficacy.     

Elevated gamma-glutamyltransferase is associated with mortality in lung transplantation for cystic fibrosis

Cystic fibrosis (CF) is a life-threatening autosomal recessive hereditary disease, affecting multiple organs. In end stage disease, lung transplantation improves the quality of life and prolongs survival. CF liver disease (CFLD) as co-morbidity develops in 8-17% of CF patients. We aimed to investigate the impact of liver injury on prognosis following lung transplantation. Thirty-one patients with CF who underwent double lung transplantation (DLTx) from 1999 to 2009 were included. Post-transplant survival, liver serum parameters as well as INR, creatinine and the MELD-Score were determined preoperatively, 1 day and 4 weeks postoperatively. Prognostic impact of liver function on outcome was analysed. Mean patient age was 25 (15-38) and post-transplant 1 year-survival was 74%, 3 years 71% and 5 years 68%. Patients were grouped according to post-transplant survival, those deceased within the first year as group I (n = 8) and patients who survived longer as group II (n = 23). Group I exhibited significantly elevated gamma-glutamyltransferase (GGT), bilirubin and reduced platelets postoperatively. Low platelet count, increased bilirubin and GGT were associated with mortality after DLTx. Prospective studies are needed to determine a potential use and clinical implications for liver function tests in patients with CF before lung transplantation.     

Cystic fibrosis is associated with an increased risk of Barrett's esophagus

BackgroundCystic fibrosis (CF) patients have increased risks of gastrointestinal cancers, including esophageal adenocarcinoma. Gastroesophageal reflux disease (GERD) is highly prevalent in CF and manifests at early ages. CF patients may be at increased risk for long-term sequelae of chronic GERD, including Barrett's esophagus (BE). We aimed to assess whether patients with CF have an increased risk of BE or related neoplasia.MethodsA matched cohort study was performed of adults with and without CF who had undergone upper endoscopy. Non-CF patients were matched in a 4:1 ratio by age, sex, year of exam, and endoscopist. Odds ratios were calculated for the association between CF and BE or related neoplasia, and multivariable logistic regression modeling was performed to adjust for matching variables and additional potential confounders.Results122 CF patients underwent endoscopy, and 488 matched controls were identified. Seven (5.7%) CF patients had BE or related neoplasia, including one GE junction adenocarcinoma. Mean age of affected CF patients was 36.0, and 85.7% had a prior solid organ transplant. The odds of BE was significantly increased in CF patients (OR 2.91, 95% CI 1.08–7.81). The risk remained significantly increased in a multivariable model including matching variables (OR 3.32, 95% CI 1.19–9.22) and in a parsimonious model (OR 2.99, 95% CI 1.06–8.42).ConclusionsAdults with CF have a 3-fold increased risk of BE or related neoplasia and appears to develop at younger ages. Consideration should be given to screening for BE in select CF patients, especially those who have undergone solid organ transplantation.     

Cystic fibrosis and portal hypertension: Distal splenorenal shunt can prevent the need for future liver transplant

BackgroundThe management of portal hypertension (PHT) in children with well compensated cirrhosis and cystic fibrosis (CF) is controversial. We present our experience with distal splenorenal shunting (DSRS) for the treatment of PHT as an alternative to liver transplantation (LT).MethodsBetween 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9) was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant).ResultsMean PELD/MELD score was significantly lower for DSRS patients than LT (3 ± 6 vs 28 ± 4, p < 0.001). All 5 DSRS patients had grade III–IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ± 1.5 cm to 4.4 ± 1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ± 48 to 91.8 ± 35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24–7.79).ConclusionsPatients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity.Type of studyCase series with no comparison group.Level of evidenceIV.     

Successful Renal Transplantation in Children With Posterior Urethral Valves

PurposeThe treatment of children with posterior urethral valve (PUV) and end-stage renal disease can be challenging. Some series have had poor outcomes after renal transplantation with an increased risk of graft dysfunction and urinary tract infections. We present our experience with a pediatric population and compare it to all the other pediatric renal transplants done at our institution.Materials and MethodsWe identified 10 patients with PUV who underwent a total of 13 renal transplants between 1990 and 2000. The comparison group included 120 transplants done in 95 patients during the same period. Cumulative allograft survival and function were recorded.ResultsOverall patient survival in the PUV group was 100%. Mean age at transplant in the PUV group was 10.0 years and mean followup was 3.9 years. Six patients underwent high proximal urinary tract diversion, while the remainder had primary transurethral valve ablation. Three patients had bladder augmentation before transplantation. Cumulative allograft survival in the PUV group at 1 and 5 years was 85% and 64%, respectively. Of the 10 patients 9 currently have functioning living related donor transplants. One patient lost 3 cadaveric donor transplants to chronic rejection. No patients lost grafts due to infection or bladder dysfunction. Mean serum creatinine of the functioning grafts was 1.1 mg/dl.ConclusionsRenal transplantation can be performed safely and effectively in patients with PUV, including those who have undergone previous proximal urinary tract diversion. Preoperative bladder management and continued monitoring of bladder and kidney function postoperatively are paramount in the preservation of allograft function.     

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.