Pulmonary Arterial Hypertension Diagnosis and Evidence-Based Treatment

by Barst, Robyn

• ISBN13: 9780470059722
• ISBN10: 0470059729
• Edition: 1st
• Format: Hardcover
• Copyright: 2008-06-23
• Publisher: Wiley

Summary

First book dedicated to this disease, previously thought to be incurable, but with the advent of new drugs, now amenable to management and a much improved prognosis for patients From the PAH Association, the leading experts in field Incorporates the latest AACP management guidelines Includes evidence-based treatment algorithms based on the recently updated ACCP Guidelines for Medical Treatment Aimed at specialists in pulmonology and cardiovascular disease, this volume provides the clinician with the most up to date information on the effective management of PAH

Author Biography

Robyn J. Barst, MD, Professor of Pediatrics (in Medicine); Director, Pulmonary Hypertension Center, Columbia University Medical Center, New York, NY, USA
Dr Barst is chair of the 'Scientific Leadership Council' of the Pulmonary Hypertension Association, a group of more than twenty internationally recognized leaders in the field of PAH. The mission of the SLC is 'to provide medical and scientific leadership for the Association by proactively facilitating the development of new knowledge about pulmonary hypertension, actively disseminating knowledge about pulmonary hypertension to medical and public audiences, and advocating and raising awareness about pulmonary hypertension. The editor and contributors are all members of the SLC, and include many well-known names including Lewis Rubin, George Simmoneau, Nazarino Galie, and David Badesch. This text is intended to be the 'official' work on the subject.
Further evidence of the standing of the contributors is the medical centers involved in the SLC, which include the Mayo Clinic, Columbia Presbyterian Medical Center, University of California San Diego, UCLA, Duke, Rush Presbyterian, University of Colorado, University of Utah, Vanderbilt, the University of Alabama at Birmingham, and the University of Pennsylvania. The SLC also has international members from Italy, Ireland, Germany, and Canada.
Dr Barst is? on the Editorial Board of Circulation, the official Journal of the American Heart Association, and for Chest, the official journal for the American Thoracic Society. Member of European Society of Cardiology Task Force for Primary Pulmonary Hypertension, Board Member of the International Society for Heart and Lung Transplantation.

Table of Contents

Preface	p. ix
Contributors	p. xi
Introduction	p. 1
Diagnosis and assessment of pulmonary arterial hypertension	p. 7
Hemodynamics of pulmonary hypertension	p. 7
Venice classification	p. 8
Overview of the diagnostic process (algorithm)	p. 10
Screening (identifying a pre-existing risk)	p. 11
Detection (discovering pulmonary hypertension)	p. 13
Definition (diagnosing the clinical context)	p. 14
Prediction (estimating prognosis)	p. 14
Principles of follow-up (evaluating and responding to outcome)	p. 16
Specific tests in the evaluation of pulmonary hypertension	p. 20
Conventional therapy in pulmonary arterial hypertension	p. 47
Calcium channel antagonists	p. 48
Diuretics	p. 52
Warfarin	p. 52
Supplemental oxygen	p. 54
Inotropic therapy	p. 55
General measures	p. 56
Conclusions	p. 57
Prostanoid treatment for pulmonary arterial hypertension	p. 61
Epoprostenol	p. 63
Treprostinil	p. 77
Iloprost	p. 80
Beraprost	p. 81
Treatment selection	p. 82
Conclusions	p. 83
Endothelin receptor antagonists in pulmonary arterial hypertension	p. 89
Bosentan	p. 92
Sitaxsentan	p. 96
Ambrisentan	p. 99
Safety and tolerability with endothelin receptor antagonists	p. 101
Conclusions	p. 102
Phosphodiesterase-5 inhibitors in pulmonary arterial hypertension	p. 105
The origins of phosphodiesterase-5 inhibitor development	p. 105
Pulmonary hypertension as a new indication for phosphodiesterase-5 inhibitor treatment	p. 108
Role of phosphodiesterase-5 in the pulmonary vasculature	p. 109
Clinical experience with sildenafil for the treatment of chronic pulmonary hypertension	p. 110
Pivotal trial and approval of sildenafil for the treatment of pulmonary arterial hypertension (SUPER-1 study)	p. 111
Other phosphodiesterase-5 inhibitors	p. 112
Combination therapy	p. 114
Potential new indications for phosphodiesterase-5 inhibitors outside pulmonary arterial hypertension	p. 116
Conclusions	p. 118
Combination therapy for pulmonary arterial hypertension	p. 127
Background	p. 127
Combination therapy to date	p. 130
Conclusions	p. 142
Interventional and surgical modalities of treatment for pulmonary arterial hypertension	p. 147
Introduction	p. 147
Atrial septostomy	p. 148
Pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension	p. 157
Lung transplantation for pulmonary hypertension	p. 161
Conclusions	p. 172
End points and clinical trial design in pulmonary arterial hypertension: Clinical and regulatory perspectives	p. 179
Introduction	p. 179
Trial design	p. 180
End points in trials of therapy for pulmonary arterial hypertension	p. 185
Conclusions	p. 197
Comparative analysis of clinical trials and evidence-based treatment algorithm for pulmonary arterial hypertension	p. 203
Introduction	p. 203
Randomized controlled studies	p. 207
Long-term continuation studies	p. 209
Combination studies	p. 213
Evidence-based treatment algorithm	p. 214
Conclusions	p. 216
Diagnosis and assessment of non-pulmonary arterial hypertension masquerading as idiopathic pulmonary arterial hypertension: Diastolic heart failure - evaluation and interactions	p. 223
Definition of heart failure	p. 223
Epidemiology	p. 224
Physiology	p. 225
Evaluation of diastolic heart failure: Invasive and noninvasive techniques	p. 226
Diagnosis and treatment of diastolic dysfunction	p. 229
Pulmonary hypertension and diastolic dysfunction	p. 230
Conclusions	p. 232
Treatment of pulmonary arterial hypertension: A look to the future	p. 237
Therapy of pulmonary arterial hypertension	p. 240
Measuring outcomes and monitoring the course of therapy	p. 243
Conclusions	p. 243
Index	p. 247
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