Pulmonary Arterial Hypertension: Diagnosis and Evidence-Based Treatment Book

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