European Respiratory Society
Lung Transplantation

Lung transplantation is a treatment option for selected patients who are not responding to maximal medical therapy, or for whom no effective medical therapy exists. This book provides a comprehensive guide to the recent advances in lung transplantation, and includes coverage of donor selection, immunosuppression, infectious and noninfectious complications, and management of bronchiolitis obliterans syndrome and post-transplant lymphoproliferative disease emerging after lung transplantation.

  • European Respiratory Society Monographs
  1. Page vii
  2. Page viii
  3. Page ix
  4. Page 1
    Abstract
    Correspondence: G.M. Verleden, University Hospital Gasthuisberg, Dept of Respiratory Medicine, Lung Transplantation Unit, 49, Herestraat, B-3000 Leuven, Belgium. E-mail: geert.verleden@uzleuven.be

    Referral for lung transplantation should be considered when the patient has an end-stage (cardio)pulmonary disease, for which no medical treatment exists or which no longer responds to maximal medical treatment, and an expected 2- to 3-yr survival of <50% and/or a New York Heart Association class III–IV level of function. Referral should be early enough so that the patient may survive the sometimes long waiting times before transplantation. This is a specific consideration in small patients with an O or B blood group. Concomitant extrapulmonary diseases should be adequately taken care of, bearing in mind that absolute contraindications for lung transplantation do exist.

  5. Page 9
    Abstract
    Correspondence: M. Delcroix, Dept of Pneumology, University Hospital of Leuven, Herestraat 49, 3000 Leuven, Belgium. E-mail: marion.delcroix@uzleuven.be

    Lung or heart–lung transplantation used to be the only treatment that offered improved survival in patients with severe pulmonary arterial hypertension (PAH). The advent of medical therapy that currently targets three pathways of importance in the pathobiology of this lethal condition has led to a major change in our approach to treatment. All patients should be referred to centres expert in the management of PAH and the newer therapeutic approaches include prostacycline analogues, endothelin receptor antagonists and phosphodiesterase 5 inhibitors.

    Each of these classes of drug has been shown to improve haemodynamics, functional status and exercise performance when used as monotherapy. Although the majority of studies are short term, extended follow-up has also provided data showing improved survival when compared with historical controls. In addition, there is a growing literature supporting the role of combining the different drug classes in a patient both in terms of safety and efficacy. These drugs have been shown to be effective for a wide range of patients including those with idiopathic or familial PAH, and PAH in association with connective tissue disease, portal hypertension, HIV and congenital heart disease.

    There has been renewed interest in the role of atrial septostomy as an adjunct therapy for selected patients who are failing on medical treatment. It can be considered as a bridge to transplantation for some patients who are deteriorating on active transplant waiting lists.

    None of the treatments above can be considered as curative; however, many patients who have pulmonary hypertension due to chronic thromboembolic disease (i.e. chronic thromboembolic pulmonary hypertension) are suitable for curative surgery in the form of thromboendarterectomy, which effectively removes the fibrotic obstruction that leads to the raised pulmonary vascular resistance in these patients.

    Although the introduction of oral drug therapy for pulmonary hypertension has apparently simplified its treatment, the complete diagnosis, assessment and management strategy for an individual patient remains complex and fully supports the delivery of care centred on expert centres.

  6. Page 25
    Abstract
    Correspondence: G.I. Snell, Lung Transplant Service, 5th Floor, Alfred Hospital and Monash University, Commercial Road, Melbourne, 3004, Australia. E-mail: g.snell@alfred.org.au

    Chronic obstructive pulmonary disease (COPD) is the most common indication for lung transplantation, although there has been a trend away from this recently. In assessing COPD patients for transplant it is also important to optimise their condition to enhance the prospects of reaching lung transplantation, enhance the lung transplantation outcomes and even potentially find a successful alternative to transplant.

    Smoking cessation and the management of associated anxiety, panic or depression are important starting points. Multidisciplinary pulmonary rehabilitation is a proven therapy. Supplemental oxygen can help endurance training and enhances pre-lung transplantation survival in hypoxaemic patients. Nasal positive pressure ventilation is not of proven benefit in stable patients, but may benefit those during exacerbations and as a physiotherapy adjunct.

    The comorbidities of COPD, in particular coronary artery disease, thoracic malignancy and osteoporosis, must be considered and managed at assessment. If possible, resistant bacterial (i.e. stenotrophomonas), fungal and mycobacterial infections should be treated pre-lung transplantation. Dynamic hyperinflation represents a possible therapeutic target, and it is approriate to consider surgical and bronchoscopic lung reduction techniques.

    In contrast to the nihilism typifying 20th century management, COPD is now recognised as a complex disease capable of significant therapeutic potential in the hands of an expert multidisciplinary team. Much can be done for the COPD patient beyond consideration of transplantation.

  7. Page 32
    Abstract
    Correspondence: C. Knoop, Dept of Chest Medicine, University Hospital, 808, Route de Lennik, 1070 Brussels, Belgium. E-mail: cknoop@ulb.ac.be

    Better care has allowed the postponement of the time-point of end-stage cystic fibrosis (CF) and the need for lung transplantation. Indeed, most CF children now have a fairly good outlook and the vast majority of today's CF lung transplant candidates are adults.

    In the absence of a curative treatment, the natural history of CF has, however, remained the same: a vicious cycle of infection and inflammation, worsening bronchiectasis and progressive airway(s) obstruction resulting, ultimately, in respiratory failure.

    The philosophy behind maximising therapy prior to lung transplantation is not very different fromp the philosophy behind CF care in general.

    The two goals are as follows: 1) to gain years of life with good or, at least, acceptable quality of life and 2) to maintain general status and vital organ function in order to not compromise a future lung transplantation.

  8. Page 46
    Abstract
    Correspondence: W.A. Wuyts, Interstitial Lung Disease Unit, University Hospitals Leuven, Leuven, Belgium. E-mail: wim.wuyts@uzleuven.be

    Idiopathic pulmonary fibrosis (IPF) is a devastating disease that carries a median survival of 3 yrs after diagnosis. It is characterised by fibroblast proliferation and extracellular matrix remodelling, resulting in irreversible lung distortion. Lung transplantation is so far the only treatment that provides a survival advantage. However, mortality of IPF patients on the waiting list is high, urging the need for timely referral for transplantation and defining novel medical therapies. Several recent trials have investigated therapies directly influencing the pathophysiological mechanisms of the disease. Although most of these trials failed to meet their primary end-points, progress has been made in understanding the disease process, and marginal trends towards improved outcome or disease progression are promising for reaching a breakthrough in the near future. Besides pharmacological treatment, extensive supportive measurements are needed to ensure optimal physical and mental strength. Oxygen therapy improves symptoms of breathlessness and quality of life. Pulmonary rehabilitation and optimal nutritional support are further important components of the management of patients with advanced IPF and will potentially complement post-transplant management. The natural course of IPF is troubled by the appearance of acute exacerbations of IPF that are often fatal. This highlights the need for timely referral for lung transplantation, since this is the only treatment that has been shown to improve survival.

  9. Page 54
    Abstract
    Correspondence: M. Carby, Royal Brompton and Harefield NHS Trust, Harefield Hospital, Hill End Road, Harefield, Middlesex UB9 6JH, UK. E-mail: m.carby@rbht.nhs.uk

    Severe lung disease often co-exists with a multiplicity of other physical and psychological issues that must be addressed if lung transplantation is to be successful. Diabetes mellitus, arterial hypertension and coronary artery disease should be treated according to relevant specialist society guidelines. Body mass index, whether too high or too low, can impact negatively on lung transplant outcome and should be addressed. Physical deconditioning, respiratory failure, pneumothorax, gastro-oesophageal reflux, impact of drug treatment and terminal care are all issues requiring consideration. Management of osteoporosis is addressed elsewhere.

    Aggressive management of comorbidities can maximise the functional status of the individual while awaiting surgery, giving the highest possible expectation of successful short- and long-term outcomes following lung transplantation.

  10. Page 62
    Abstract
    Correspondence: E. Kochetkova, Service de Chirurgie Thoracique, Nouvel Hôpital Civil/NHC, 1, place de l'Hôpital Civil – BP N 426, 67091 Strasbourg Cedex, France. E-mail: zkochetkova@mail.ru

    Transplantation-related osteoporosis (OP) causes significant morbidity in the growing population of survivors of organ transplantation. Increasing attention has been given to OP before and after transplantation of solid organs (i.e. kidneys, liver and heart). This problem is of particular importance in candidates for lung transplantation: a high incidence of osteopenia and OP was registered in patients with different types of end-stage pulmonary diseases.

    Spontaneous fractures appear as systemic complications of pulmonary diseases in pre- and post-transplant periods, which significantly deplete the quality of life. Pathogenesis of the pre- and post-transplant osteopenic syndrome includes multiple complex factors, namely age, sex, duration of disease, ventilation disorders, hypoxaemia, smoking, poor nutrition, underweight, hypokinesia, intestinal calcium absorption decrease and vitamin D deficiency. Immunosuppressive drugs, especially steroids, have a negative effect on bone metabolism. Cyclosporine and tacrolimus lead to severe osteopenia in rats, but the skeletal toxicity of calcineurin inhibitors in humans is less clear.

    Preventive strategies include minimising steroid exposure and implementing therapies to counter the increases in bone resorption and decrease in bone formation. Antiresorptive agents, especially bisphosphonates, may delay or stop early bone loss and reduce fracture rates. A role for other agents in the transplant setting requires further investigation. Obviously, antiresorptive treatment must be started during the pre-transplant period to minimise the number of osteoporotic complications and allow for optimised immunosuppressive therapy in patients after lung transplantation.

  11. Page 81
    Abstract
    Correspondence: P. Botha, Dept of Cardiopulmonary Transplantation, Freeman Hospital, Newcastle Upon Tyne, NE7 7DN, UK. E-mail: P.Botha@ncl.ac.uk

    The critical shortage of donor lungs suitable for transplantation significantly limits the number of potential recipients that can benefit from this therapy.

    Although the majority of studies undertaken to evaluate the efficacy of commonly used donor selection criteria are typically retrospective in nature, a number of important studies have emerged. These have demonstrated that donor selection practice in the majority of centres worldwide remains unnecessarily strict.

    In some series, it has been shown that current lung transplant activity could be doubled in many regions if more aggressive selection practices were employed. It is also becoming apparent that this can be achieved without any detrimental effect on either short- or long-term outcome. However, many aspects of lung donor selection have not been the subject of rigorous scientific scrutiny and, as such, practice remains based on experience (and often most recent experience). It should therefore be a priority for the international lung transplant community to further investigate the appropriateness of selection practices and allow the maximal safe utilisation of this precious resource.

    We review the available literature and summarise our own practice regarding the selection of a potential lung donor.

  12. Page 88
    Abstract
    Correspondence: J.M.A. Smits, Eurotransplant International Foundation, P.O. Box 2304, 2301 CH Leiden, The Netherlands. E-mail: jsmits@eurotransplant.nl

    Lung donor recognition and treatment could be further improved. In 2007, according to Eurotransplant (an organ-exchange organisation comprising Austria, Belgium, Croatia, Germany, Luxembourg, Slovenia and the Netherlands), only 22% of all donors were actual lung donors. A large difference in lung donation rates also exists between the Eurotransplant countries: in 2007, in Belgium, 8.49 lung donors per million population were utilised for transplantation compared with 3.09 lung donors per million population population in Germany.

    During the last decade, more efficient use of lung donors has been made. Donor utilisation rate, i.e. the conversion of reported to actual donor, increased from 45% in 1999 to 56% in 2007. Among the actual lung donors, the proportion of donors where both lungs were used for transplantation increased from 87% in 1999 to 91% in 2007. The median donor age of a utilised lung donor increased by 7 yrs to 43 yrs in 2007.

    In order to increase lung donor utilisation rates, utilisation of extended-criteria lung donors could be increased. At present, lung donor acceptance policies for lung donors with specific conditions (donors who died from intoxication, donors with a malignancy, donors with a positive virology (hepatitis B-virus surface antigen and/or anti-hepatitis C virus)) differ within the Eurotransplant countries. The adapted Oto lung donor quality score is a useful tool for donor recognition. Additonally, the organ utilisation rate can be applied to gauge the suitability for transplantation of a lung donor. Eurotransplant has four mechanisms in place in order to ensure that the highest degree of allocation effectiveness is achieved with every reported lung donor.

  13. Page 104
    Abstract
    Correspondence: D.E.M. Van Raemdonck, Dept of Thoracic Surgery, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium. E-mail: dirk.vanraemdonck@uzleuven.be

    The widespread application of lung transplantation is limited by the lack of suitable organs from brain-dead donors resulting in long waiting times for listed patients with a substantial risk of dying prior to transplantation. To overcome this critical organ shortage, many transplant programmes worldwide have begun to explore alternative pathways to increase their lung donor pool.

    Several groups have shown that lungs from donors not matching the ideal criteria for lung donation can be successfully transplanted with good long-term outcome. Nevertheless, caution needs to be exercised when transplanting less optimal lungs in recipients with an increased operative risk. Successful techniques to downsize the pulmonary graft such as peripheral (segmental) resection, lobe transplantation and pulmonary bipartitioning have been developed in order to transplant grafts from larger donors into smaller recipients. This is of specific interest for children and small women facing longer waiting times.

    The use of lobes from living donors is also an option mainly in patients with cystic fibrosis receiving the lower lobes from two different donors. Improved long-term outcome with a lower incidence of bronchiolitis obliterans syndrome has been reported. The number of teams reporting successful transplantation of lungs recovered from a donor after cardiac arrest has increased over the last few years. Most organs still come from controlled non-heart-beating donors, but lungs from donors after unexpected cardiac death can also be successfully transplanted with reported results comparable to heart-beating donors. Ex vivo reperfusion for reconditioning of initially rejected donor lungs appears to be a promising development to increase the lung donor yield.

    Finally, there is still a long gap to bridge before pulmonary xenotransplantation will become clinical reality to help all those in need waiting for new lungs to become available.

  14. Page 131
    Abstract
    Correspondence: M. Strueber, Dept of Cardiothoracic, Transplant and Vascular Surgery, Hanover Medical School, Carl-Neuberg-Strasse 1, 30625 Hanover, Germany. E-mail: strueber.martin@mh-hannover.de

    Primary graft dysfunction (PGD) still represents the primary cause of death in the peri-operative period after lung transplantation. The clinical hallmarks are lung oedema with a reduction of oxygenation and compliance of the transplanted lung. The incidence is still significant, in the range of 20%, and significantly impairs survival.

    Numerous attempts have been made to improve both lung preservation and peri-operative management to avoid PGD. Indeed, progress has been made both in our understanding and in preventive measures. However, as the multifactorious nature of PGD is now well established, no singular therapy is available to avoid this phenomenon. In addition, criteria for patient selection, as well as for acceptance of donor grafts, have been expanded by most centres. This has led to an increase in the lung transplant activities and also increases the peri-operative risk for PGD. Thus, it seems that the progress that has been made in avoiding PGD has been counteracted by the use of extended criteria for graft acceptance and also for patient selection.

    The role of brain death of the donor in inducing an inflammatory response to the graft is now understood. Preservation solutions of the extracellular type containing low potassium levels are well established. The inhibition of surfactant and a potential role of surfactant replacement therapy are discussed. The peri-operative management of fluid restrictions and the use of extracorporeal devices for lung support in high-risk patients both prior to and after transplantation are also addressed.

    Although much effort has already been made, the field of PGD still deserves the attention of future investigations to both improve patient outcome and also to increase the availability of donor grafts in the future.

  15. Page 147
    Abstract
    Correspondence: M. Iversen, Division of Lung Transplantation 2142, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, 2100 Copenhagen, Denmark. E-mail: maiv@rh.dk

    Immunosuppression in adult lung transplantation is mostly based on experience from other types of solid organ transplantation, however, many results may not be extrapolated directly to lung transplantation.

    Calcineurin inhibitors (CNIs), such as cyclosporine and tacrolimus, are considered essential in lung transplantation and, at the present time, CNI-free protocols are not recommended because of insufficient immunosuppressive effect. Available evidence does not demonstrate any clinically significant difference between the use of cyclosporine or tacrolimus or between the use of azathioprin or mycophenolate mofetil. The use of induction therapy is not considered essential and is used by approximately half of all lung transplant centres.

    The role of cell proliferation inhibitors, such as sirolimus and everolimus, is yet to be established, but will probably be in combination with CNIs in reduced doses.

    Short- and long-term side-effects due to CNI treatment are common and often lead to changes in dose or type of drug. In long-term survivors, toxicity from immunosuppressive drugs becomes increasingly important.

  16. Page 169
    Abstract
    Correspondence: F.K. Gould, Microbiology Dept, Freeman Hospital, High Heaton, Newcastle Upon Tyne, NE7 7DN, UK. E-mail: kate.gould@nuth.nhs.uk

    Infections play an important role throughout the clinical course of lung transplantation and a thorough knowledge of the various infections, their clinical presentation, timing after transplantation and treatment is a prerequisite for the management of the lung transplant patient.

    Infection management starts at candidate selection, as recipients can be colonised with multi-resistant bacteria or fungi. Infections may also be acquired from the donor or after transplantation.

    Timing is very important when evaluating a transplant patient with an infection, as different infections appear at different intervals after transplantation. Early infections are typically bacterial (such as wound infections or ventilator-associated pneumonia); later on, viral reactivations of herpes viruses and community-acquired pathogens, including respiratory viruses, may be implicated. The infections range from ‘common’ infections to opportunistic infections. Luckily, for some infections, such as cytomegalovirus and Pneumocystis carinii pneumonia, prophylaxis has become standard. Re-colonisation with Pseudomonas can be detected with routine cultures. Most infections are recognised, even in the context of immunosuppression, but classical symptoms might be masked.

    The role of infection (viral pseudomonal and fungal) as a risk factor for the development of chronic rejection is still under debate, but evidence is emerging that it might contribute to chronic allograft dysfunction.

  17. Page 177
    Abstract
    Correspondence: G. Massard, Service de Chirurgie Thoracique, Pôle de Pathologie Thoracique et Groupe de Transplantation Pulmonaire, Hôpitaux Universitaires de Strasbourg, 67091 Strasbourg, France. E-mail: Gilbert.Massard@chru-strasbourg.fr

    Despite continuously improving results, lung transplantation still suffers from a high complication rate. The most important noninfectious and nonimmunological complication is impaired healing of the airway anastomosis. It may present as acute and early dehiscence, or covered necrosis leading to granulation with formation of stenosis. Although it concerns 15–20% of transplants, <3% of these complications are lethal, and only half of them require bronchoscopic procedures. Rare complications are stenosis or thrombosis of the vascular anastomosis, bleeding disorders, parietal problems, phrenic nerve dysfunction and acute abdominal complications. Exceptionally, the primary disease may recur in the transplanted lung, as described in some orphan indications. There are some specific complications in the native lung after single-lung transplantation. In patients with emphysema, there is a risk of acute hyperinflation during the post-operative period, often related to acute allograft dysfunction; in the medium term, progressive inflation of the native lung might compromise function of the graft. In former smokers, there is a real risk for lung cancer in the native lung, which carries an overall bad prognosis.

  18. Page 197
    Abstract
    Correspondence: G.M. Verleden, University Hospital Gasthuisberg, Dept of Respiratory Medicine, Lung Transplantation Unit, 49, Herestraat, B-3000 Leuven, Belgium. E-mail: geert.verleden@uzleuven.be

    Lung transplantation is currently an accepted treatment modality for selected patients with end-stage lung disease.

    Although the survival rates have improved in recent years, long-term survival remains inferior compared with other solid-organ transplantations, such as kidney, heart and liver. The main reason is the development of chronic allograft dysfunction, characterised by a progressive and irreversible decline in the forced expiratory volume in 1 s. This process is histologically manifested as obliterative bronchiolitis (OB) and is considered to be the consequence of chronic rejection.

    As a result of the difficulties in obtaining good pathological specimens, a clinical grading system, called bronchiolitis obliterans syndrome (BOS), has been introduced, divided into five categories, depending on the severity of airflow obstruction. Extensive research efforts have attempted to unravel the pathophysiology of OB/BOS; however, this remains unclear at the present time.

    Recently, however, newer insights into OB/BOS have been proposed, with the identification of at least two different phenotypes of OB/BOS. One of these phenotypes, characterised by neutrophilic inflammation of the airways, seems to respond to the addition of azithromycine, a neo-macrolide antibiotic, whereas the other phenotype does not. Overall, treatment options for the latter form are almost nonexistent and retransplantation may be the only option in carefully selected patients.

  19. Page 212
    Abstract
    Correspondence: L.J. Dupont, Division of Respiratory Medicine, University Hospital Gasthuisberg, 49 Herestraat, B-3000 Leuven, Belgium. E-mail: lieven.dupont@uz.kuleuven.be

    Alloimmune-mediated injury directed against endothelial and epithelial structures has been thought to be the underlying cause of bronchiolitis obliterans syndrome (BOS) or chronic rejection after lung transplantation. However, non-alloimmune inflammation, including airway colonisation and retrograde aspiration secondary to gastro-oesophageal reflux (GOR), have also been implicated as potential contributors to the development of BOS.

    Data suggest a link between both GOR and persistent airway colonisation with Pseudomonas and Aspergillus spp. and the development of BOS after lung transplantation. The possible pathways in which GOR or airway colonisation may alter immunogenicity or contribute to chronic rejection will need to be elucidated, although it appears that GOR may interact with innate immunity. Further prospective studies are needed to confirm these data and determine the most effective approach to prevent lung injury related to GOR or colonisation in order to improve long-term lung transplant outcomes.

  20. Page 226
    Abstract
    Correspondence: E.A.M. Verschuuren, Lung Transplant Team, Dept of Pulmonary Diseases, University Medical Centre Groningen, P.O. Box 30.001, 9700 RB, Groningen, The Netherlands. E-mail: e.a.m.verschuuren@int.umcg.nl

    Immunosuppression includes the risk of complications of an impaired immune system, including malignancies and post-transplant proliferative disease, which have become major complications after lung transplantation. Nonmelanoma skin cancer has the highest incidence, but the incidence of other solid organ tumours is also increased.

    The oncogenic effect of immunosuppressive agents, as well as oncogenic viruses and impaired immune control, are important causative factors of cancer after transplantation. The level and duration of immunosuppression are strongly related to the development of cancer.

    The best known-virus induced malignancy is post-transplant lymphoproliferative disease (PTLD), of which the relation with the Epstein–Barr virus (EBV) is generally accepted. PTLD accounts for all lymphomas after transplantation, with a very diverse clinical course and treatment.

    A standard work-up should be carried out to determine whether a patient has the relatively benign form that will respond to reduction of immunosuppression alone or a very malignant B- or T-cell lymphoma that will need (poly-)chemotherapy. Some centres will use pre-emptive therapy (reduction of immunosuppression) based on EBV DNA load to prevent PTLD, but its role needs to be established.

  21. Page 238
    Abstract
    Correspondence: W. van der Bij, Groningen Lung Transplant Program, Dept of Internal Medicine, University Hospital Groningen, Hanzeplein 1, 9713 GZ Groningen, The Netherlands. E-mail: w.van.der.bij@int.umcg.nl

    Nowadays, lung transplantation is an established therapeutic option for patients with various end-stage lung diseases, such as chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), pulmonary fibrosis and pulmonary hypertension. Despite disappointing results in the 1970s and 1980s, progress and refinement over the last two decades in both surgical techniques and medical management, including especially the introduction of cyclosporine A, have been impressive.

    Patient survival has improved significantly over time, with a current overall survival half-life of 5.2 yrs, and >7 yrs for those who have survived ≥1 yr. In uncomplicated cases, near-normalisation of pulmonary function is the rule, with >80% of survivors at 1, 3, 5 and 10 yrs reporting no activity limitations. Exercise performance is usually ∼50% of predicted, suggesting extrapulmonary causes. Quality of life improves significantly in all domains, including employment status. A major limitation of long-term survival is still chronic allograft dysfunction, which is histologically represented by an obliterative bronchiolitis and functionally by bronchiolitis obliterans syndrome. The forfeit of improved survival is a longer exposure to immunosuppression with its inherent morbidity, such as an increased risk of infection, drug toxicity and malignancies.

    While the establishment of standard maintenance immunosuppressive and anti-microbial regimens was the major concern some 25 yrs ago, the future challenge will be to develop individualised drug regimens guided by immunological and biological markers in order to prevent rejection and prolong survival, while limiting morbidity and medication side-effects of lung-transplant recipients.

  22. Page 251
    Abstract
    Correspondence: C. Benden, Division of Pulmonary Medicine, University Hospital Zurich, Raemistrasse 100, CH-8091 Zurich, Switzerland. E-mail: christian_benden@yahoo.de

    Lung transplantation has evolved as an accepted therapy in selected children with end-stage lung disease, offering prolongation of life and an improved quality of life. To date, >1,500 lung transplants in children have been performed.

    Paediatric lung transplantation presents a specific challenge with regards to the child’s underlying disease, a challenging surgical approach, the effects of immunosuppressants, the impact of infections on the developing immune system and somatic growth.

    Cystic fibrosis is the main indication for transplant; however, indications vary considerably by age group. To achieve maximal survival benefit, early referral, careful patient selection and appropriate timing of listing are crucial. The predicted life expectancy without transplantation has to be balanced with the expected post-transplant survival, and waiting list time must also be taken into account.

    Post-transplant, careful monitoring of the graft function is essential. Infectious complications are a major cause for morbidity and mortality early post-transplant. Bronchiolitis obliterans is the main cause for mortality by 5 yrs post-transplant. More than half of the surviving children develop bronchiolitis obliterans syndrome by 5 yrs post-transplant. Outcome after paediatric lung transplantation is reported to be similar to adults, aiming for a good long-term survival with normal growth and development, and a good quality of life.

  23. Page 266
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