Pulmonary Vascular Disorders


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Series: Pulmonary and Respiratory Diseases and Disorders

BISAC: MED079000

Pulmonary vascular disorders are a complex and fascinating group of diseases. The aim of this book is to exhaustively review the genetics, physiology, diagnosis, management, and treatment of pulmonary vascular disorders with the purpose of helping general physicians and specialists in internal medicine, cardiology, pneumology, immunology, rheumatology, and hematology to identify and assess patients with vascular pulmonary disorders. Special emphasis has been placed upon pulmonary hypertension, pulmonary fibrosis, and pulmonary embolism as well as on pulmonary vascular involvement in immune-mediated diseases, chronic obstructive and advanced pulmonary disease, and sleep apnea. Additional topics that are covered in this book include pulmonary vascular injury occurring in alpha-1 antitrypsin deficiency, congenital heart disease and pregnancy. The text is supported by figures and tables to help the reader understand mechanisms of pulmonary vascular disorders in different clinical settings.

Table of Contents


Part I. General Principles

Chapter 1. The Genetic Basis of Pulmonary Arterial Hypertension
(Emilia M. Swietli, MD and Nicholas W. Morrell, MD – Department of Medicine, University of Cambridge School of Clinical Medicine, Addenbrooke’s Hospital, Cambridge, Biomedical Campus, Cambridge, UK)

Chapter 2. Physiology Related to Pulmonary Vascular Disorders: An Overview
(Roberto G. Carbone, MD and Francesco Puppo, MD – Department of Internal Medicine, University of Genoa, Genoa, Italy)

Chapter 3. Immunology in Pulmonary Vascular Disorders: Focus on Pulmonary Arterial Hypertension
(Frédéric Perros, MD, Florent Soubrier, MD, Fabrice Antigny, MD, Rozenn Quarck, MD, Marc Humbert, PhD, David Montani, MD and Sylvia Cohen-Kaminsky, MD – Université Paris-Saclay, AP-HP, INSERM UMR_S 999, Service de Pneumologie et Soins Intensif Respiratoires, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France, et al.)

Chapter 4. Pathology in Pulmonary Vascular Disorders: The Past, Present, and Future
(Rubin M. Tuder, MD – Program in Translational Lung Research Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Colorado School of Medicine, CO, USA)

Chapter 5. Imaging of Pulmonary Hypertension
(Erica Altschul, MD, Priti Mehla, MD, Stephen Machnicki, MD, Roxanne Sulica, MD, Varun Shah, MD, Nick Hill, MD and Suhail Raoof, MD – Lung Center, Lenox Hill Hospital, Western Region Pulmonary Service, Northwell Health, New York, NY, USA, et al.)

Part II. Cardiopulmonary Diseases

Chapter 6. Diagnosis of Idiopathic Pulmonary Arterial Hypertension
(Benjamin Nguyen, MBBS, Marc Humbert, PhD and Edmund M. Lau, PhD – Royal Alfred Hospital, University of Sydney, Australia, et al.)

Chapter 7. Pulmonary Vascular Involvement in Idiopathic Pulmonary Fibrosis
(Mada Ghanem, MD and Bruno Crestani, MD – Université de Paris, Inserm, U1152, Service de Pneumologie A, Hôpital Bichat, Paris, France)

Chapter 8. Vascular Injury in Chronic Obstructive Pulmonary Disease
(Isabel Blanco, MD and Joan A. Barberà, MD – Hospital Clinic, IDIBAPS, University of Barcelona, Barcelona, Spain)

Chapter 9. Pulmonary Hypertension in Obstructive Sleep Apnea and Interstitial Lung Disease
(Yang Zhou, MD, Katherine E. Cox-Flaherty, MD and James R. Klinger, MD – Brown University Department of Molecular Microbiology and Immunology, et al.)

Chapter 10. Pulmonary Vascular Complications in Congenital Heart Diseases
(Julie Wacker, MD and Maurice Beghetti, MD – Pediatric Cardiology Unit, University Children’s Hospital HUG, Pulmonary Hypertension Program HUG, Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique (CURCCCP), University of Geneva and Lausanne, Geneva and Lausanne, Switzerland)

Chapter 11. Cor Pulmonale in 2020
(Robert Naeije, MD – Department of Pathophysiology, Université Libre de Bruxelles, Bruxelles, Belgium)

Chapter 12. Diagnosis and Management of Acute Pulmonary Embolism
(Michael McMahon, MD, Shari Barnett Brosnahan, MD and Lisa K. Moores, MD – Department of Medicine, Tripler Army Medical Center, Honolulu, HI, USA, et al.)

Chapter 13. Chronic Thromboembolic Pulmonary Hypertension: Causes, Diagnosis, and Therapy
(Estefania Oliveros, MD, Victor F. Tapson, MD and William Auger, MD – Department of Medicine; Temple University Hospital, Philadelphia, PA, USA, et al.)

Chapter 14. Acute Pulmonary Embolism: Classification, Risk Stratification and Critical Aspects of Therapy
(Victor F. Tapson, MD – Division of Pulmonary and Critical Care, U.S. Pulmonary Embolism Consortium, Cedars-Sinai Medical Center, Los Angeles, CA, USA)

Chapter 15. New Drugs in Idiopathic Pulmonary Arterial Hypertension
(Robert N. Channick, MD – Pulmonary and Critical Care Division, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA)

Part III. Systemic Diseases

Chapter 16. Sarcoidosis Associated Pulmonary Hypertension
(Robert P. Baughman, MD and Elyse E. Lower, MD – Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA)

Chapter 17. Pulmonary Vasculitis in ANCA-associated and Goodpasture’s Disease
(Kathryn A. Lai, MD and Namita Sood, MD – McGovern Medical School, University of Texas Health Science Center, Houston, TX, USA, et al.)

Chapter 18. Pulmonary Hypertension in Rheumatoid Arthritis
(Joshua J. Solomon, MD, Roberto G. Carbone, MD and Francesco Puppo, MD – Autoimmune Lung Center, National Jewish Health, Denver, CO, USA, et al.)

Chapter 19. Pulmonary Vascular Injury in Systemic Sclerosis: Diagnosis and Treatment
(Francesco Puppo, MD, Simone Negrini, PhD, and Roberto G. Carbone, MD – Department of Internal Medicine, University of Genoa, Genoa, Italy)

Chapter 20. Lung Involvement in Systemic Lupus Erythematosus and Disseminated Intravascular Coagulation
(Assaf Monselise, PhD, Roberto G. Carbone, MD and Francesco Puppo – Department of Internal Medicine, Tel Aviv University, Israel, et al.)

Chapter 21. Other Causes of Disseminated Intravascular Coagulation
(Keith C. Meyer, MD – UW Lung Transplant & Advanced Pulmonary Disease Program, Section of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, University of Wisconsin School of Medicine and Public Health)

Chapter 22. Pulmonary Vascular Disorders and Alpha-1 Antitrypsin Deficiency
(Roberto G. Carbone, MD and Giovanni Bottino, MD – Department of Internal Medicine, University of Genoa, Genoa, Italy)

Chapter 23. Pulmonary Hypertension in Pregnancy
(Christine Y. Zhou, DO, Ralph J. Panos, MD and Jean M. Elwing, MD – Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, OH, USA, et al.)

Part IV. Advanced Lung Diseases

Chapter 24. Pulmonary Vascular Disease and Transplant in Advanced Lung Diseases
(Peter Yau, MD, Tasnim Lat, DO, Oksana Shlobin, MD, Adriel Malave, MD and Deborah J. Levine, MD – Texas A&M Health Sciences Center, Division of Pulmonary, Critical Care & Sleep Medicine, Baylor Scott & White Health Temple, TX, USA, et al.)



“It is my pleasure to write these lines in support of the publication of this book addressing the vascular disorders of the pulmonary system. The editors have been able to convene the best specialists to address issues that range from the genetics, through the primary diseases of the vessels and ending with advanced pulmonary diseases. It is a field that has evolved very rapidly, where an authoritative review can help place together the incremental information accrued over the last decades. I commend the editors for their effort and hope this book will make the impact it deserves.” – Bartolome Celli, M.D. Professor of Medicine, Harvard Medical School

“As knowledge of pulmonary vascular disease rapidly evolves, current summaries of this information from recognized experts in the field become increasingly necessary. This book brings together leaders from around the world to provide a unique resource. They have synthesized previous and current research addressing key areas of the disease state, in manner that will move the field forward. The authors and editors should be commended for this work in a period of exceptional challenge for everyone involved in healthcare.” – David B. Badesch, MD Professor of Medicine, Divisions of Pulmonary Sciences and Critical Care Medicine, and Cardiology, Clinical Director, Pulmonary Hypertension Center, University of Colorado, Denver, CO, USA

“There have been enormous advances in the understanding and management of pulmonary vascular disorders; pulmonary hypertension has been transformed from a fatal disease to a treatable condition with the introduction of effective new treatments. This book, edited by Roberto Carbone, Francesco Puppo, and Victor Tapson, brings together some of the world experts in pulmonary vascular disease to give a comprehensive overview and update of these important diseases, with discussions on their genetics, pathophysiology, diagnosis and treatment. As well as comprehensively discussing pulmonary hypertension and pulmonary thromboembolism, the book covers the involvement of the pulmonary vasculature in other lung diseases, such as interstitial lung diseases, chronic obstructive pulmonary disease and sleep apnoea, as well as the involvement of the pulmonary vasculature in systemic disorders, including rheumatoid arthritis and other immunological diseases, cardiovascular diseases and in pregnancy. This seminal book will be invaluable to physicians from several specialities and to researchers.” – Peter Barnes FRS, FMedSci, Imperial College London

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