Table of Contents
Introduction Claude Lenfant vii
Preface ix
Contributors xiii
Section 1 Classification, Epidemiology, Pathology, Pathogenesis and Genetics
1 Updated Clinical Classification of Pulmonary Hypertension Ivan M. Robbins Gérald Simonneau 1
2 Epidemiology of Pulmonary Arterial Hypertension Marc Humbert 10
3 Pathology of Pulmonary Hypertension Peter Dorfmüller 20
4 Pathogenesis of Pulmonary Hypertension Norbert F. Voelkel Lori Sweeney Herman Bogaard 40
5 Animal Models of Pulmonary Hypertension Laurence Dewachter Robert Naeije 59
6 Genetics of PAH Eric D. Austin James E. Loyd John A. Phillips III 83
Section 2 Imaging and Diagnosis of Pulmonary Hypertension
7 Imaging of Pulmonary Hypertension Dominique Musset Sophie Maître 95
8 Echocardiography of Pulmonary Arterial Hypertension Adam Torbicki Anna Fijalkowska 122
9 Diagnosis and Hemodynamic Assessment of Pulmonary Arterial Hypertension Rajan Saggar Rajeev Saggar John A. Belperio David A. Zisman Joseph P. Lynch III Jamil Aboulhosn 132
Section 3 Clinical Variants of Pulmonary Hypertension
10 Idiopathic, Familial, and Anorexigen-Associated Pulmonary Arterial Hypertension Jerome Le Pavec Marc Humbert 150
11 Pulmonary Arterial Hypertension Complicating Connective Tissue Diseases Paul M. Hassoun 161
12 Pulmonary Arterial Hypertension in Congenital Heart Disease Daniel S. Levi Victoria Scott Jamil Aboulhosn 176
13 Pulmonary Arterial Hypertension and Human Immunodeficiency Virus Infection Bruno Degano Olivier Sitbon 196
14 Portopulmonary Hypertension Michael J. Krowka 207
15 Pulmonary Hypertension Complicating Schistosomiasis Caio J. C. S. Fernandes Carlos Jardim Rogério Souza 214
16 Pulmonary Hypertension in Sickle Cell Disease Mark T. Gladwin 222
17 Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis David Montani 237
18 Pulmonary Hypertension in Chronic Obstructive Pulmonary Disease Emmanuel Weitzenblum Ari Chaouat 250
19 Pulmonary Hypertension Complicating Interstitial Lung Disease David A. Zisman John A. Belperio Rajan Saggar Rajeev Saggar Michael C. Fishbein Joseph P. Lynch III 264
20 Pulmonary Hypertension in Chronic Mountain Sickness Dante Penaloza Francisco Sime 292
21 Chronic Thromboembolic Pulmonary Hypertension William R. Auger Peter F. Fedullo 305
Section 4 Medical Treatment of Pulmonary Arterial Hypertension
22 Pulmonary Arterial Hypertension: Conventional Therapy Olivier Sitbon 325
23 Medical Treatment of PAH: Prostacyclins Paul R. Forfia Vallerie V. McLaughlin 338
24 Phosphodiesterase Type 5 Inhibitors Nazzareno Galiè 350
25 Medical Treatment of Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists Kristina Kemp Marc Humbert 355
26 Medical Treatment of PAH: Combination Therapy, Novel Agents, Future Directions, and Current Recommendations Lewis J. Rubin Sean P. Gaine 367
27 Goal-Oriented Therapy in Pulmonary Arterial Hypertension Marius M. Hoeper 377
Section 5 Atrioseptostomy and Surgery for Pulmonary Hypertension
28 Atrial Septostomy for the Treatment of Severe Pulmonary Hypertension Julio Sandoval Jorge Caspar Tomás Pulido 388
29 Surgical Management of Pulmonary Hypertension Philippe G. Dartevelle Elie Fadel Sacha Mussot Dominique Fabre Olaf Mercier Marc Humbert Gérald Simonneau 401
30 The Selection and Timing of Transplantation for Patients with Pulmonary Arterial Hypertension Paul A. Corris 412
Section 6 Difficult Pulmonary Hypertension
31 Pulmonary Arterial Hypertension and Pregnancy Xavier Jaïs Laura Claire Price Florence Parent Marc Humbert 422
32 Pediatric Pulmonary Hypertension Cecile Tissot Maurice Beghetri 434
33 Practical Management of Pulmonary Arterial Hypertension in the Intensive Care Unit Benjamin Sztrymf Marc Humbert 455
Section 7 Endpoints and Clinical Trials
34 End Points and Clinical Trial Design in Pulmonary Arterial Hypertension: Clinical and Regulatory Perspectives Andrew J. Peacock 462
Index 477
