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Evolving Knowledge and New Horizons Ganesh Raghu Roberto G Lung Transplantation Evolving Knowledge and New Horizons Ganesh Raghu Roberto G. Carbone Editors 123 Lung Transplantation Ganesh Raghu • Roberto G. Carbone Editors Lung Transplantation Evolving Knowledge and New Horizons Editors Ganesh Raghu Roberto G. Carbone Division of Pulmonary Department of Internal Medicine Critical Care, and Sleep Medicine University of Genoa Department of Medicine Genoa University of Washington Italy Seattle, WA USA ISBN 978-3-319-91182-3 ISBN 978-3-319-91184-7 (eBook) https://doi.org/10.1007/978-3-319-91184-7 Library of Congress Control Number: 2018950336 © The Editor(s) (if applicable) and The Author(s) 2018 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Printed on acid-free paper This Springer imprint is published by the registered company Springer International Publishing AG part of Springer Nature The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland Epigraph Difficulty in breathing with limited life span to breathing easy with extended life following lung transplantation. —Ganesh Raghu v I dedicate this book to all the donors and recipients of lung transplantation and to the caregivers and families of pre- and post-lung transplant patients. —Ganesh Raghu The capacity to help others is a blessing. I dedicate this book to those who donated life to others and to those who, by their heroic decision to donate the organs of their loved ones, gifted new life to others. —Roberto G. Carbone Preface Management of advanced or end-stage nonmalignant lung diseases is always challenging to the pulmonary specialist as the path of the breath of life for patients with lung diseases comes to an end with no further medical treat- ments to extend life. The frustrations and sense of despair that the patient, their loved ones, families, friends, caregivers, and physicians are confronted with are significant. While many patients unfortunately succumb to advanced lung diseases, new horizons have opened up for patients suffering from end-stage lung disease, thanks to pioneers including Cooper, Pearson, and Viganò, by mak- ing lung transplantation a reality to these patients, who are otherwise healthy. Since the advent of lung transplantation, nearly 6000 patients have received lung transplantations between 1988 and 1995, increasing progres- sively to 17,715 bilateral lung transplantations between 2005 and 2010, with a statistically significant (p < 0.0001) smaller risk of rejection com- pared to the preceding period considered. Indeed, lung transplantation has evolved into standard of care for these patients, with improved quality of life and enhanced survival for patients with idiopathic pulmonary fibrosis (IPF), cystic fibrosis, etc. Indeed, lung transplantation is a potential option for the patient who has reached advanced stages, but, unfortunately, it is not an option for all patients who have reached advanced stages and for whom there are no further medical treatment interventions to stabilize their lung problems. The general pulmon- ologist needs to be aware of the indications and contraindications of lung transplantation as well as of post-lung transplant management, risks, compli- cations, and outcomes in order to be able to help patients understand the complexity and benefits of lung transplantation as well as be prepared for the anticipated outcomes. Pulmonologists also must help patients improve and/or maintain their physical stability and overall fitness as possible while coping with advanced stages of diseases such as IPF, chronic obstructive pulmonary disease (COPD), cystic fibrosis, pulmonary hypertension, pulmonary sar- coidosis, interstitial pneumonia associated with connective tissue diseases such as systemic sclerosis, rheumatoid arthritis, and polymyositis, and other orphan lung diseases. This is the first book to discuss all aspects of lung transplantation and is intended to equip the general pulmonologist/physician worldwide with all the necessary tools, awareness, and knowledge to help patients with management of advanced stages, preparation for lung transplantation, and care post-lung ix x Preface transplantation. This book also serves as a clinical guide to refer appropriate patients early for consideration for lung transplantation worldwide. We have organized the chapters into four broad parts and have leading global experts who have written on the different topics pertinent to medical, surgical, psychological, and social aspects of lung transplantation. Additionally, we have summarized the history of lung transplantation and discussed the immunological aspects and epigenetic changes with vascular injury after lung transplantation. The first part discusses eligibility criteria including specific evaluation, timing for listing, indications and contraindications for lung transplantation, pretransplant considerations, psychosocial patient evaluation, and lung allo- cation score, in combination with the impact of new revisions and successive outcome. A particular emphasis is given to lung transplantation with regard to COPD, cystic fibrosis, and interstitial lung diseases with pulmonary fibrosis. Issues related to the donor lung with detailed consideration of immunol- ogy, physiology, epigenetics, and surgical treatment are discussed in the sec- ond part. The third part discusses medical management of the lung transplantation recipient including cellular and humeral rejection, prevention of viral and nonviral infection, lung allograft dysfunction, and complications such as bronchiolitis obliterans pneumonia, gastro-esophageal reflux, and essential topics including bronchoscopy, imaging, and lung rejection pathology. Finally, the last part highlights directions for the future to bridge the gaps in the field of lung transplantation. We are very grateful to all the leading experts in the field for agreeing to discuss their expertise in their chapters and for their contribution to this book dedicated to lung transplantation. Ganesh Raghu Roberto G. Carbone Seattle, WA, USA Genoa, Italy Contents Part I General Principles and Considerations for Lung Transplantation 1 The History of Lung Transplantation .................... 3 Andrea Maria D’Armini, Valentina Grazioli, and Mario Viganò 2 Single- and Bilateral Lung Transplantation: Indications, Contraindications, Evaluation, and Requirements for Patients to Be Considered Eligible ................... 17 Gerard J. Meachery and Paul A. Corris 3 Pretransplant Considerations in Patients with Obstructive Lung Disease: COPD, Lymphangioleiomyomatosis, Cystic Fibrosis, and Non-CF Bronchiectasis .................... 41 Shahnaz Ajani and Robert M. Kotloff 4 Pretransplant Considerations in Patients with Pulmonary Fibrosis .............................. 57 Roberto G. Carbone, Assaf Monselise, Keith M. Wille, Giovanni Bottino, and Francesco Puppo 5 Pulmonary Vascular Disease: Specific Evaluation and Timing for Listing Eligible Patients to Receive Lung Transplantation and Management of the Pre-lung Transplant Patient ...................... 71 Alison S. Witkin and Richard N. Channick 6 Psychosocial Evaluation of the Patient Considered for Lung Transplant .................................. 85 Thomas M. Soeprono and Ganesh Raghu 7 Prognostic Markers and the LAS for Lung Transplantation: Impact of New Revisions for Successful Outcome .......... 93 M. Patricia George and Matthew R. Pipeling xi xii Contents Part II The Donor Lung and the Lung Transplant Recipient 8 Management of the Donor and Recipient: Surgical Management ................................. 113 Andrea Mariscal and Shaf Keshavjee 9 Immunology in Lung Transplantation ................... 139 Idoia Gimferrer and Karen A. Nelson 10 Physiologic and Epigenetic Changes with Pulmonary Vascular Injury After Lung Transplantation .............. 161 Steven Kenneth Huang, Roberto G. Carbone, and Giovanni Bottino Part III The Lung Transplant Recipient: Medical Management 11 Medical Management of the Lung Transplant Recipient: Extrapulmonary Issues ................................ 185 Erika D. Lease and Ganesh Raghu 12 Management of Cellular and Humoral Rejection: Prevention, Diagnosis, and Treatment .............................. 195 Erika D. Lease and Ganesh Raghu 13 Pathology of Lung Rejection: Cellular and Humoral Mediated ............................... 209 Anja C. Roden and Henry D. Tazelaar 14 Prevention and Treatment Regimens for Non-viral
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