Pulmonary circulation

750 Pages·2011·17.19 MB·English

Pulmonary Circulation
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Pulmonary Circulation:
Diseases and their Treatment
THIRD EDITION
Andrew J Peacock
Professor of Medicine, Consultant Respiratory
Physician and Director, Scottish Pulmonary Vascular Unit,
Regional Heart and Lung Centre, Golden Jubilee National Hospital,
Glasgow, UK
Robert Naeije
Professor of Physiology and Medicine and Consultant,
Department of Cardiology, Erasme Academic Hospital,
The Free University of Brussels, Belgium
Lewis J Rubin
Emeritus Professor of Medicine and Emeritus Director,
Division of Pulmonary and Critical Care Medicine,
University of California, San Diego School of Medicine,
La Jolla, California, USA
First published in Great Britain in 2004 by Hodder Arnold.
Second edition 2004.
This third edition published in 2011 by Hodder Arnold, an imprint of
Hodder Education, Hodder and Stoughton Ltd, a division of Hachette UK
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© 2011 Hodder & Stoughton Ltd
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ISBN-13 978-0-340-981-924
1 2 3 4 5 6 7 8 9 10
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Dedication
To my wife Jila and my children Leila, Johnnie and Vita who have put up with so much over the years and never questioned my love for
science and medicine even though their own interests lay elsewhere.
Andrew Peacock
To my family for their support, and to our patients, who serve as a constant reminder of the importance of our commitment.
Lewis Rubin
We would like to thank our colleagues from around the world who have been an inspiration to this book and in many cases contributors
to it. Whether we work in Respiratory Medicine or Cardiology, we all share the view that the connection between the heart and
lungs is important.
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Contents
Colour plates appear between pages 344 and 345
Contributors xi
Foreword xvi
Preface xvii
Preface to the second edition xviii
Abbreviations xix
A note on references xxiii
PART 1 THE FUNCTION OF THE NORMAL PULMONARY CIRCULATION & RIGHT HEART
1 Pulmonary vascular function 3
Robert Naeije
2 Right heart function 16
Anna R Hemnes and Hunter C Champion
PART 2 PATHOPHYSIOLOGY & PATHOLOGY OF PULMONARY VASCULAR DISEASE
3 Pathology of pulmonary vascular diseases 29
Peter Dorfmüller
4 Hypoxic pulmonary vasoconstriction and hypertension 46
Andrea Olschewski
5 Pulmonary vascular remodeling and pathobiology of pulmonary hypertension 59
Nicholas W Morrell
PART 3 CLINICAL ASSESSMENT OF PULMONARY HYPERTENSION: DIAGNOSIS & MANAGEMENT
6 Clinical features 81
Andrew J Peacock and Colin Church
7 Imaging: Echocardiography 100
Marcin Kurzyna and Adam Torbicki
8 Imaging: Chest radiography, ventilation/perfusion scintigraphy and computed tomography 115
Michael Sproule
9 Imaging: Emerging modalities (MR, PET and others) 128
Anton Vonk-Noordegraaf
10 Exercise testing and pulmonary hypertension: Six-minute walk testing and cardiopulmonary exercise testing 138
Daniel Dumitrescu and Ronald J Oudiz
11 Blood biomarkers 146
Christopher Rhodes, Vahitha Abdul-Salam, John Wharton and Martin R Wilkins
12 Cardiac catheterization of patients with pulmonary hypertension 159
Todd M Bull, Bertron M Groves and David B Badesch
13 An integrated approach to the diagnosis of pulmonary hypertension 170
Robert P Frantz and Michael D MCGoon
viii Contents
PART 4 DISORDERS ASSOCIATED WITH PULMONARY HYPERTENSION
14 Updated clinical classification of pulmonary hypertension 197
David Montani and Gerald Simonneau
15.1 Idiopathic and heritable pulmonary hypertension: Introduction to pathophysiology and clinical aspects 207
Kelly Chin, Fernando Torres and Lewis J Rubin
15.2 Genetics of pulmonary arterial hypertension 212
Eric D Austin, James E Lloyd and John H Newman
15.3 Pulmonary arterial hypertension associated with connective tissue diseases 223
Paul M Hassoun
15.4 Pulmonary hypertension related to appetite suppressants 236
Olivier Sitbon, Marc Humbert and Gérald Simonneau
15.5 Pulmonary hypertension associated with portal hypertension 245
Philippe Hervé, Laurent Savale, Robert Naeije, Gérald Simonneau, Marc Humbert and Olivier Sitbon
15.6 Pulmonary arterial hypertension associated with HIV infection 251
Rudolf Speich
15.7 Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts 258
Maurice Beghetti
15.8 Hemolysis-associated pulmonary hypertension in sickle cell disease and thalassemia 271
Claudia R Morris and Mark T Gladwin
15.9 Pulmonary hypertension in pregnancy 288
Laura Price, Xavier Jaïs and Marc Humbert
PART 5 DRUG AND NON DRUG TREATMENT OF PULMONARY HYPERTENSION
16.1 Conventional medical therapies: General measures, supportive treatments and calcium channel blockers 303
Nazzareno Galiè, Alessandra Manes, Massimiliano Palazzini and Enri Leci
16.2 Prostacyclins: Intravenous, subcutaneous, inhaled and oral 313
Vallerie Mclaughlin and Melvyn Rubenfire
16.3 Inhaled nitric oxide and nitric oxide donors 326
Roberto F Machado
16.4 Endothelin receptor antagonists 335
Richard N Channick
16.5 The nitric oxide and cyclic guanosine monophosphate pathway 343
Colin Church and Andrew Peacock
16.6 Combination therapy for pulmonary arterial hypertension 353
Steven M Kawut and Harold I Palevsky
16.7 Stem cell therapy 361
Mark L Ormiston, David W Courtman and Duncan J Stewart
16.8 Novel anti-proliferative strategies 368
Soni Savai Pullamsetti, Friedrich Grimminger and Ralph Theo Schermuly
PART 6 SURGICAL TREATMENT OF PULMONARY HYPERTENSION
17.1 Lung transplantation 381
Paul A Corris
17.2 Atrial septostomy and other interventional procedures 391
Julio Sandoval and Jorge Gaspar
17.3 Pulmonary endarterectomy 405
Eckhard Mayer
18 An integrated approach to the treatment of pulmonary arterial hypertension 413
Marius M Hoeper
PART 7 PULMONARY HYPERTENSION DUE TO CAPILLARY OR POST CAPILLARY DISEASE
19.1 Pulmonary hypertension secondary to left heart failure 425
Marco Guazzi
Contents ix
19.2 Pulmonary veno-occlusive disease 435
Barbara A Cockrill and Charles A Hales
19.3 Pulmonary capillary hemangiomatosis 447
David Langleben
PART 8 P ULMONARY HYPERTENSION ASSOCIATED WITH DISORDERS OF THE RESPIRATORY SYSTEM
AND/OR HYPOXEMIA
20.1 Pulmonary hypertension due to chronic hypoxic lung disease: Chronic obstructive pulmonary disease and 457
interstitial lung disease
Joan Albert Barberà, Isabel Blanco and Sandra Pizarro
20.2 Pulmonary hypertension in obstructive sleep apnea 472
Kathleen Sarmiento and Steven M Scharf
20.3 An integrated approach to the treatment of pulmonary hypertension related to hypoxic lung diseases 479
Ari Chaouat, Emmanuel Gomez, Matthieu Canuet, Christine Selton-Suty, François Chabot and Emmanuel Weitzenblum
PART 9 OTHER IMPORTANT CAUSES OF PULMONARY HYPERTENSION
21 Schistosomiasis and others in group 5 491
Rogério Souza, André Hovnanian, Caio Júlio César Dos Santos Fernandes and Carlos Viana Poyares Jardim
PART 10 A CUTE AND CHRONIC PULMONARY THROMBOEMBOLISM, PULMONARY VASCULAR TUMORS AND
PULMONARY VASCULITIS
22.1 Diagnosis of acute pulmonary thromboembolism 503
Arnaud Perrier and Henri Bounameaux
22.2 Treatment of acute pulmonary embolism 517
Victor F Tapson
22.3 Chronic thromboembolic pulmonary hypertension 528
Diana Bonderman and Irene M Lang
22.4 Pulmonary vascular tumors 538
Kim M Kerr
22.5 Pulmonary vasculitis 544
Karina Keogh and Sean P Gaine
PART 11 PULMONARY HYPERTENSION IN PEDIATRICS
23.1 Idiopathic pulmonary arterial hypertension in children 559
Dunbar Ivy
23.2 Persistent pulmonary hypertension of the newborn: Pathophysiology and treatment 572
Steven H Abman and Robin H Steinhorn
PART 12 PULMONARY CIRCULATION IN CRITICAL CARE
24.1 Effects of mechanical ventilation on the pulmonary circulation 587
Michael R Pinsky
24.2 Effects of lung injury on the pulmonary circulation 607
Laura Price, TW Evans and S John Wort
24.3 Pharmacological management of pulmonary circulation in critically ill patients 625
Horst Olschewski
PART 13 PULMONARY CIRCULATION IN SPECIAL ENVIRONMENTS
25.1 High altitude pulmonary hypertension 637
Dante Penaloza