Lung Transplantation Editedby NicholasR.Banner RoyalBromptonandHarefieldNHSTrustand NationalHeartandLungInstitute,ImperialCollegeLondon JuliaM.Polak ImperialCollegeLondon and MagdiH.Yacoub HarefieldResearchFoundationandImperialCollegeLondon CAMBRIDGE UNIVERSITY PRESS Cambridge, New York, Melbourne, Madrid, Cape Town, Singapore, São Paulo Cambridge University Press The Edinburgh Building, Cambridge CB2 8RU, UK Published in the United States of America by Cambridge University Press, New York www.cambridge.org Information on this title: www.cambridge.org/9780521651110 © Cambridge University Press 2003 This publication is in copyright. Subject to statutory exception and to the provisions of relevant collective licensing agreements, no reproduction of any part may take place without the written permission of Cambridge University Press. First published 2003 This digitally printed version 2007 A catalogue record for this publication is available from the British Library ISBN 978-0-521-65111-0 hardback ISBN 978-0-521-03677-1 paperback Every effort has been made in preparing this book to provide accurate and up-to-date information which is in accord with accepted standards and practice at the time of publication. Nevertheless, the authors, editors and publisher can make no warranties that the information contained herein is totally free from error, not least because clinical standards are constantly changing through research and regulation. The authors, editors and publisher therefore disclaim all liability for direct or consequential damages resulting from the use of material contained in this book. Readers are strongly advised to pay careful attention to information provided by the manufacturer of any drugs or equipment that they plan to use. Cambridge University Press has no responsibility for the persistence or accuracy of URLs for external or third-party Internet websites referred to in this publication, and does not guarantee that any content on such websites is, or will remain, accurate or appropriate. To Ursula and Mary N.R.B. To Daniel J.M.P. To Marianne M.H.Y. Contents Listofcontributors ix Preface xv PartI Pulmonarydisease 1 Primarypulmonaryhypertension: pathophysiologyandtherapy 3 StuartRich 2 Geneticsofpulmonaryhypertension 11 RichardC.TrembathandRachelHarrison 3 Pathologyofpulmonaryhypertension 19 AnneE.BishopandJuliaM.Polak 4 Pulmonaryhypertensionandtherightventricle 29 NorbertF.Voelkel,RubinM.Tuder,CarlyneCool,Mark Geraci,RobertQuaifeandMichaelBristow 5 Emphysema 39 GordonL.Snider 6 Alpha-1-antitrypsindeficiency 54 DuncanC.S.Hutchison 7 Bronchiectasis 61 MaryT.Keogan 8 Cysticfibrosis 71 MargaretE.Hodson vii viii Contents 9 Diffuselungdisease 85 20 Infectiouscomplications 250 R.M.duBois KeithMcNeil,JulietFowerakerandTimWreghitt 10 Explantpathology 96 21 Cytomegalovirusinfection 261 SusanStewart NicholasR.Banner 22 Imaging 274 PartII Lungtransplantation R.JaneChambers 11 Overview 113 23 Transplantpathology 294 MagdiH.YacoubandEmmaJ.Birks MargaretM.Burke 12 Patientselectionandindicationsforlung 24 Haematology 326 tranplantation 120 DavidCummins JanetR.Maurer 25 Psychology 337 13 Singleandbilaterallungtransplantation 132 ClaireN.HallasandJoWray JohnDark 26 Thecurrentstatusoflungtransplantation 353 14 Combinedheartandlungtransplantation 141 AllanR.Glanville RosemaryRadley-Smith,AsgharKhaghaniand NicholasR.Banner 15 Anaesthesiaandintensivecare 158 PartIII Futuredirections MichaelJ.BoscoeandShaneJ.George 27 Tissueengineering 367 16 Medicalmanagement 176 LarryL.Hench,JulianR.Jones,RubiaF.S.Lenzaand PaulA.Corris WanderL.V.Vasconcelos 17 Immunologicalmechanismsofgraft 28 Xenotransplantation 374 injury 185 AgnesM.Azimzadeh,CarstenSchroederandRichardN. MarleneL.RoseandIanV.Hutchinson PiersonIII 18 Pharmacologicalimmunosuppression 205 29 Theartificiallung 386 NicholasR.BannerandHaifaLyster BrackG.HattlerandWilliamJ.Federspiel 19 Chroniclungallograftdysfunction 243 MarshallI.Hertz Index 399 Contributors AgnesM.Azimzadeh ResearchAssistant DepartmentofCardiothoracicSurgery VanderbiltUniversityMedicalCenter 2986TheVanderbiltClinic Nashville TN37323-5734 USA Presentaddress AssistantProfessorofSurgery UniversityofMaryland CardiacSurgeryN4W94 22SouthGreeneSt. BaltimoreMD21201-1595 USA [email protected] NicholasR.Banner ConsultantinCardiologyandTransplantMedicine RoyalBromptonandHarefieldNHSTrust HarefieldHospital Harefield Middlesex UB96JH UK [email protected] EmmaJ.Birks SpecialistRegistrarinCardiology HarefieldResearchFoundation HarefieldHospital Harefield Middlesex UBP6JH UK [email protected] ix x Contributors AnneE.Bishop CarlyneCool SeniorLecturer AssistantProfessor TissueEngineeringandRegenerativeMedicineCentre DepartmentofPathology ImperialCollegeFacultyofMedicine UniversityofColoradoHealthSciencesCenter ChelseaandWestminsterHospital UniversityofColarado 369FulhamRoad HealthScienceCenter London 4200East9thAvenue#C310 SW109NH Denver [email protected] CO80220–3706 USA MichaelJ.Boscoe ConsultantAnaesthetist PaulA.Corris HarefieldHospital ProfessorofThoracicMedicine Harefield TheFreemanHospital Middlesex HighHeaton UB96JH NewcastleuponTyne UK NE77DN [email protected]@aol.com UK [email protected] MichaelBristow ProfessorofMedicine DavidCummins DivisionofCardiology ConsultantHaematologist UniversityofColoradoHealthSciencesCenter HarefieldHospital UniversityofColarado Harefield HealthScienceCenter Middlesex 4200East9thAvenue#C310 UB96JH Denver UK CO80220–3706 [email protected] USA MargaretM.Burke JohnDark ConsultantHistopathologist ProfessorofCardiothoracicSurgery HarefieldHospital RegionalCardiothoracicCentre Harefield TheFreemanHospital Middlesex NewcastleuponTyne UB96JH NE77DN UK UK [email protected] [email protected] R.JaneChambers R.M.duBois ConsultantRadiologist ProfessorofRespiratoryMedicine HarefieldHospital RoyalBromptonHospital Harefield SydneyStreet Middlesex London UB96JH SW36NP UK UK [email protected] [email protected] Contributors xi WilliamJ.Federspiel ClaireN.Hallas DepartmentofChemicalEngineering,Departmentsof HealthPsychologist SurgeryandBioengineering HarefieldHospital McGowanInstituteofRegenerativeMedicine Harefield UniversityofPittsburgh Middlesex PittsburghPA15219 UB96JH USA UK [email protected] [email protected] JulietFoweraker RachelHarrison ConsultantMicrobiologist ResearchFellow MicrobiologyLaboratory DivisionofMedicalGenetics PapworthHospital AdrianBuilding PapworthEverard UniversityofLeicester Cambridgeshire UniversityRoad CB38RE Leicester UK LE17RH [email protected] [email protected] ShaneJ.George BrackG.Hattler ConsultantinAnaesthesiaandIntensiveCare ProfessorofSurgery HarefieldHospital ArtificialLungProgram Harefield UniversityofPittsburgh Middlesex 200LothropStreet UB96JH C-700 UK Pittsburgh [email protected] PA15213 USA MarkGeraci [email protected] AssociateProfessor DivisionofPulmonarySciencesandCriticalCare LarryL.Hench Medicine ProfessorofMaterials UniversityofColoradoHealthSciencesCenter CentreforTissueRegenerationandRepair UniversityofColarado ImperialCollegeofScienceTechnologyandMedicine HealthScienceCenter PrinceConsortRoad 4200East9thAvenue#C310 London Denver SW72BP CO80220–3706 UK USA [email protected] AllanR.Glanville MarshallI.Hertz AssociateProfessorofMedicine ProfessorofPulmonary/CriticalCareMedicine StVincent’sHospital PulmonaryMedicine/LungTransplantProgram Xavier4 UniversityofMinnesota VictoriaStreet 420DelawareStreetSE Darlinghurst 276MayoMailRoom Sydney Minneapolis NSW2010 MN55455 Australia USA [email protected] [email protected] xii Contributors MargaretHodson A.Khaghani ProfessorofRespiratoryMedicine ClinicalDirectorofTransplantation DepartmentofCysticFibrosis HarefieldHospital RoyalBromptonHospital Harefield SydneyStreet Middlesex London UB96JH SW36NP UK UK [email protected] [email protected] RubiaF.S.Lenza DuncanC.S.Hutchison ResearchAssociate ConsultantinRespiratoryMedicine FederalUniversityofMinasGerais King’sCollegeHospital DepartmentofMetallurgicalandMaterialsEngineering DenmarkHill RuaEspiritoSanto,35–200Andar London 30160–030BeloHorizonte SE59RS MG UK Brazil [email protected] HaifaLyster IanV.Hutchinson TransplantPharmacist ProfessorofImmunology HarefieldHospital ManchesterUniversity Harefield OxfordRoad Middlesex Manchester UB96JH M139PL UK UK [email protected] [email protected] JanetR.Maurer JulianR.Jones MedicalDirector ResearchAssociate LIFESOURCETransplantNetwork DepartmentofMaterials CIGNAHealthCare ImperialCollegeofScienceTechnologyandMedicine 900CottageGroveRoad PrinceConsortRoad Bloomfield London CT SW72BP USA UK [email protected] KeithMcNeil MaryT.Keogan ConsultantPhysician ConsultantImmunologist PapworthHospital PapworthHospital PapworthEverard PapworthEverard Cambridge Cambridge CB38RE CB38RE UK UK Presentaddress Presentaddress DepartmentofThoracicMedicine ConsultantImmunologist PrinceCharlesHospital BeaumontHospital RodeRoad DepartmentofImmunology Chermside BeaumontRoad Brisbane Dublin9 Queensland4032 Ireland Australia [email protected] keith–[email protected]