BOOK
Pulmonary Arterial Hypertension, An Issue of Clinics in Chest Medicine, E-Book
(2013)
Additional Information
Book Details
Abstract
Guest editor Terence K. Trow has assembled an expert team of authors on the topic of Pulmonary Arterial Hypertension. Articles include: Epidemiology of Pulmonary Arterial Hypertension, Pathology of Pulmonary Hypertension, Genetics of Pulmonary Arterial Hypertension, Diagnosis of Pulmonary Arterial Hypertension, Pulmonary Hypertension Owing to Left Heart Disease, Pulmonary Hypertension due to Lung Disease and/or Hypoxia, Pulmonary Arterial Hypertension Associated with Congenital Heart Disease, World Health Organization Group 5 Pulmonary Hypertension, and more!
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | Cover | ||
| Pulmonary Arterial Hypertension\r | i | ||
| Copyright\r | ii | ||
| Contributors | iii | ||
| Contents | vii | ||
| Clinics In Chest Medicine\r | xii | ||
| Preface | xiii | ||
| Epidemiology of Pulmonary Arterial Hypertension | 619 | ||
| Key points | 619 | ||
| Introduction | 619 | ||
| Changes in the Classification of the Pulmonary Hypertensive Diseases | 619 | ||
| Idiopathic PAH | 621 | ||
| Prognostic Factors in IPAH | 622 | ||
| Heritable PAH | 625 | ||
| PAH Associated with Specific Conditions | 627 | ||
| Collagen vascular diseases | 627 | ||
| HIV | 628 | ||
| Portal hypertension | 628 | ||
| Anorectic agents | 629 | ||
| Hemoglobinopathies | 630 | ||
| Pulmonary veno-occlusive disease | 630 | ||
| Summary | 631 | ||
| References | 631 | ||
| Pathology of Pulmonary Hypertension | 639 | ||
| Key points | 639 | ||
| Introduction | 639 | ||
| Normal pulmonary circulation | 640 | ||
| Intimal lesions | 643 | ||
| Medial remodeling | 645 | ||
| Adventitial remodeling | 646 | ||
| Veins in PAH | 646 | ||
| Inflammation in PAH | 647 | ||
| Methodological approaches to sampling | 647 | ||
| Relevance of animal models | 648 | ||
| Summary | 649 | ||
| References | 649 | ||
| Genetics of Pulmonary Arterial Hypertension | 651 | ||
| Key points | 651 | ||
| Pulmonary arterial hypertension | 651 | ||
| Family pedigrees | 652 | ||
| Autosomal Dominant Inheritance | 652 | ||
| Incomplete Penetrance | 652 | ||
| Variable Age of Onset | 652 | ||
| Common Ancestry | 653 | ||
| Disease-causing mutations | 653 | ||
| Bone Morphogenetic Protein Receptor Type II Gene Mutations | 653 | ||
| BMPR2 Mutations in IPAH | 654 | ||
| Some BMPR2 Mutations Are Missed by DNA Sequencing | 654 | ||
| BMPR2 Mutations Are Widespread and Diverse, and May Guide Effective Interventions | 654 | ||
| BMPR2 Mutations Cause Cellular Dysfunction Beyond the Pulmonary Circulation | 655 | ||
| How Do Patients with BMPR2 Mutations Differ from Those with IPAH? | 656 | ||
| Activin Receptor–Like Kinase-1 Gene Mutations | 656 | ||
| Endoglin Gene Mutations | 656 | ||
| Mothers Against Decapentaplegic 9 Gene Mutations | 656 | ||
| Caveolin Gene Mutations | 657 | ||
| Acid-Dependent Potassium Channel Gene | 657 | ||
| New locus for susceptibility to PAH identified through genome-wide association analysis | 657 | ||
| Prevalence of HPAH | 657 | ||
| BMPR2 mutations in APAH | 658 | ||
| PAH Associated with Anorexigen Ingestion | 658 | ||
| PAH Associated with Congenital Systemic-to-Pulmonary Shunts | 658 | ||
| PAH Associated with Connective Tissue Diseases, Viral Infection, or Portal Hypertension | 658 | ||
| Pulmonary Veno-occlusive Disease | 659 | ||
| Pulmonary Capillary Hemangiomatosis | 659 | ||
| Genetic counseling and testing | 659 | ||
| Monitoring of asymptomatic BMPR2 mutation carriers | 659 | ||
| Pharmacogenetics of PAH | 659 | ||
| Calcium channel blockers and vasoreactivity | 660 | ||
| Summary | 660 | ||
| Acknowledgments | 660 | ||
| References | 660 | ||
| Diagnosis of Pulmonary Arterial Hypertension | 665 | ||
| Key points | 665 | ||
| Introduction | 665 | ||
| Patient history | 666 | ||
| Physical examination | 666 | ||
| Electrocardiography | 666 | ||
| Chest radiography | 667 | ||
| Pulmonary function testing and hypoxemia assessment | 668 | ||
| Doppler echocardiography | 668 | ||
| Excluding thromboembolic disease | 673 | ||
| Serologic testing | 673 | ||
| MRI | 674 | ||
| Exercise testing | 675 | ||
| Cardiac catheterization | 675 | ||
| Summary | 676 | ||
| References | 676 | ||
| Pulmonary Hypertension Owing to Left Heart Disease | 683 | ||
| Key points | 683 | ||
| Introduction: nature of the problem | 683 | ||
| Epidemiology | 683 | ||
| Diagnosis | 684 | ||
| History and physical examination | 684 | ||
| Laboratory testing/imaging | 684 | ||
| Electrocardiogram | 684 | ||
| Brain Natriuretic Peptide | 684 | ||
| Chest Radiography | 684 | ||
| Pulmonary Function Testing | 685 | ||
| Echocardiography | 685 | ||
| Echocardiography in pulmonary hypertension | 685 | ||
| Echocardiography in left heart disease | 685 | ||
| Echocardiography in pulmonary hypertension owing to left heart disease | 686 | ||
| MRI | 686 | ||
| MRI in pulmonary hypertension | 686 | ||
| MRI in left heart disease | 687 | ||
| MRI in pulmonary hypertension owing to left heart disease | 687 | ||
| Integrating the data prior to catheterization | 687 | ||
| Cardiac catheterization | 687 | ||
| Importance of Cardiac Catheterization | 687 | ||
| Basics of Cardiac Catheterization | 688 | ||
| Waveforms and Interpretation | 688 | ||
| Cardiac Output Measurement | 688 | ||
| Provocative Maneuvers | 689 | ||
| Pitfalls of Cardiac Catheterization | 691 | ||
| Treatment of PH owing to LHD | 691 | ||
| Summary | 692 | ||
| References | 692 | ||
| Pulmonary Hypertension due to Lung Disease and/or Hypoxia | 695 | ||
| Key points | 695 | ||
| Introduction | 695 | ||
| Epidemiology and prevalence of PH | 696 | ||
| Idiopathic pulmonary fibrosis | 696 | ||
| COPD | 697 | ||
| Sarcoidosis | 697 | ||
| Nonspecific interstitial pneumonitis | 697 | ||
| Lymphangioleiomyomatosis | 697 | ||
| Pulmonary Langerhans cell histiocytosis | 698 | ||
| PH complicating sleep apnea | 698 | ||
| Overlap syndromes | 698 | ||
| Pathogenesis of PH in lung disease/hypoxia | 699 | ||
| Screening for PH in patients with parenchymal lung diseases | 699 | ||
| Treatment | 700 | ||
| Summary and future directions | 702 | ||
| References | 702 | ||
| Pulmonary Arterial Hypertension Associated with Congenital Heart Disease | 707 | ||
| Key points | 707 | ||
| Introduction | 707 | ||
| Epidemiology and pathophysiology of APAH-CHD | 708 | ||
| Anatomic and physiologic classification | 708 | ||
| Clinical classification | 708 | ||
| PAH associated with unrestrictive systemic to pulmonary shunts | 710 | ||
| Interpretation of hemodynamic data | 710 | ||
| Eisenmenger syndrome | 711 | ||
| Management strategies for ES | 713 | ||
| Targeted therapies for ES | 713 | ||
| Prostanoids | 713 | ||
| ERAs | 713 | ||
| PDE5 Inhibitors | 714 | ||
| Lung and Heart Lung Transplantation | 714 | ||
| Pulmonary hypertension in the univentricular heart: the failing Fontan | 714 | ||
| Summary | 715 | ||
| References | 715 | ||
| Portopulmonary Hypertension | 719 | ||
| Key points | 719 | ||
| Introduction | 719 | ||
| Definition of PoPH | 719 | ||
| Epidemiology and outcomes | 721 | ||
| Pathology | 722 | ||
| Pathophysiology and pathogenesis | 722 | ||
| Clinical presentation and evaluation | 725 | ||
| General Considerations | 725 | ||
| Evaluation Specific to PoPH | 725 | ||
| Treatment | 727 | ||
| Conventional Therapy | 727 | ||
| PAH-Specific Therapies | 728 | ||
| Prostacyclin analogues | 728 | ||
| Endothelin receptor antagonists | 728 | ||
| Phosphodiesterase-5 inhibitors | 728 | ||
| Combination therapy | 730 | ||
| Novel Therapeutic Approaches | 730 | ||
| Summary | 730 | ||
| Liver Transplantation | 730 | ||
| Summary | 731 | ||
| References | 731 | ||
| Pulmonary Hypertension Associated with Chronic Hemolytic Anemia and Other Blood Disorders | 739 | ||
| Key points | 739 | ||
| Introduction | 739 | ||
| Hemoglobinopathies | 739 | ||
| Sickle Cell Disease | 740 | ||
| β-Thalassemia | 743 | ||
| Red blood cell membrane disorders | 745 | ||
| Paroxysmal Nocturnal Hemoglobinuria | 745 | ||
| Hereditary Spherocytosis | 745 | ||
| Hereditary Stomatocytosis | 746 | ||
| Chronic myeloproliferative disorders | 746 | ||
| Splenectomy | 747 | ||
| Summary | 747 | ||
| References | 747 | ||
| World Health Organization Group 5 Pulmonary Hypertension | 753 | ||
| Key points | 753 | ||
| Introduction | 753 | ||
| Pathogenesis of group 5 PH | 754 | ||
| Overview of disease entities associated with group 5 PH | 755 | ||
| Group 5.1: Hematologic Disorders | 755 | ||
| PH in patients with myeloproliferative diseases (MPDs) | 755 | ||
| PH in patients after splenectomy | 756 | ||
| Group 5.2: Systemic Disorders | 757 | ||
| Sarcoidosis-associated PH (SAPH) | 757 | ||
| PH in pulmonary langerhans cell histiocytosis (PLCH) | 761 | ||
| PH in lymphangioleiomyomatosis (LAM) | 762 | ||
| PH in neurofibromatosis type 1 (NF1) | 762 | ||
| PH in pulmonary vasculitis | 763 | ||
| Group 5.3: Metabolic Disorders | 763 | ||
| PH in glycogen storage disease (GSD) | 763 | ||
| PH in gaucher disease (GD) | 764 | ||
| PH associated with thyroid disorders | 765 | ||
| Group 5.4: Other Disorders | 766 | ||
| PH in patients with chronic renal failure (CRF) on dialysis | 766 | ||
| PH in fibrosing mediastinitis (mediastinal fibrosis) | 767 | ||
| PH from tumor emboli | 768 | ||
| PAH-specific pulmonary vasodilator therapy for group 5 PH | 769 | ||
| PH in Patients with Hematologic Disorders | 769 | ||
| PH in Patients with Systemic Disorders | 769 | ||
| PH in Patients with Metabolic Disorders | 770 | ||
| PH in Patients with Other Disorders | 770 | ||
| Summary | 770 | ||
| References | 770 | ||
| Chronic Thromboembolic Pulmonary Hypertension | 779 | ||
| Key points | 779 | ||
| Introduction and definition | 779 | ||
| Epidemiology | 780 | ||
| Pathobiology of CTEPH | 781 | ||
| Clinical presentation | 782 | ||
| Evaluation of chronic thromboembolic disease | 783 | ||
| General Diagnostics | 783 | ||
| Chest radiography | 783 | ||
| Pulmonary function tests | 783 | ||
| Transthoracic echocardiogram | 784 | ||
| Lung ventilation-perfusion scan | 784 | ||
| Imaging of Pulmonary Vasculature | 784 | ||
| CT pulmonary angiography | 784 | ||
| MRI of the pulmonary vessels | 785 | ||
| Conventional pulmonary angiography | 786 | ||
| Cardiac Catheterization | 786 | ||
| Treatment | 786 | ||
| Surgical Approach to Chronic Thromboembolic Disease | 786 | ||
| Preoperative assessment | 786 | ||
| Pulmonary thromboendarterectomy | 787 | ||
| Nonsurgical Approach to Chronic Thromboembolic Disease | 788 | ||
| Medical therapy | 788 | ||
| Percutaneous pulmonary angioplasty | 790 | ||
| Summary | 791 | ||
| References | 792 | ||
| Standard Nonspecific Therapies in the Management of Pulmonary Arterial Hypertension | 799 | ||
| Key points | 799 | ||
| Introduction | 799 | ||
| Oxygen therapy | 800 | ||
| Diuretics | 801 | ||
| Digoxin therapy | 801 | ||
| Anticoagulation | 802 | ||
| CCBs | 805 | ||
| Summary | 805 | ||
| References | 806 | ||
| Oral Therapies for Pulmonary Arterial Hypertension | 811 | ||
| Key points | 811 | ||
| Introduction | 811 | ||
| Endothelin receptor antagonists | 811 | ||
| ET-1 Production | 812 | ||
| Endothelin Receptors | 812 | ||
| Clinical data | 813 | ||
| Ambrisentan | 813 | ||
| Bosentan | 813 | ||
| Safety considerations in endothelin receptor antagonist therapy | 814 | ||
| Phosphodiesterase Inhibitors | 815 | ||
| Sildenafil in PAH | 816 | ||
| Experimental pulmonary hypertension | 816 | ||
| Acute effects of sildenafil on human PAH | 816 | ||
| Chronic effects of sildenafil on human PAH | 817 | ||
| PDE5 inhibition with sildenafil in other forms of pulmonary hypertension | 817 | ||
| Tadalafil | 817 | ||
| Overview of Available Oral Therapies: Comparisons | 819 | ||
| Summary | 821 | ||
| References | 822 | ||
| Parenteral and Inhaled Prostanoid Therapy in the Treatment of Pulmonary Arterial Hypertension | 825 | ||
| Key points | 825 | ||
| Introduction | 825 | ||
| Pathophysiologic basis for prostacyclin use | 826 | ||
| Intravenous Epoprostenol | 826 | ||
| Pharmacokinetics | 826 | ||
| Drug delivery | 826 | ||
| Clinical trial data: randomized and observational | 827 | ||
| Dosing/Dose titration | 828 | ||
| Adverse effects | 828 | ||
| SC Treprostinil | 829 | ||
| Pharmacokinetics | 829 | ||
| Drug delivery | 829 | ||
| Clinical trial data: randomized and observational | 829 | ||
| Dosing/Dose titration | 830 | ||
| Adverse effects | 831 | ||
| Intravenous Treprostinil | 831 | ||
| Clinical trial data: randomized and observational | 831 | ||
| Dosing/Dose titration | 832 | ||
| Adverse effects | 832 | ||
| The Clinical Perspective | 832 | ||
| Criteria for Initiating the Parenteral Prostanoids | 833 | ||
| Dosing and cost-related issues | 833 | ||
| Inhaled Iloprost | 834 | ||
| Pharmacokinetics | 834 | ||
| Drug delivery | 834 | ||
| Clinical trial data | 835 | ||
| Adverse effects | 835 | ||
| Cost | 835 | ||
| Inhaled Treprostinil | 836 | ||
| Pharmacokinetics | 836 | ||
| Drug delivery | 836 | ||
| Clinical trial data | 836 | ||
| Adverse effects | 836 | ||
| Cost | 836 | ||
| Summary | 837 | ||
| References | 837 | ||
| Combination Therapy in Pulmonary Arterial Hypertension | 841 | ||
| Key points | 841 | ||
| Prostacyclin analogues + endothelin receptor antagonists | 842 | ||
| Small or Nonrandomized Trials and Case Series | 842 | ||
| Randomized Trials | 843 | ||
| Prostacyclin analogues + PDE5-Is | 847 | ||
| Small or Nonrandomized Trials and Case Series | 847 | ||
| Randomized Trials | 848 | ||
| Oral combination therapy | 848 | ||
| Small or Nonrandomized Trials and Case Series | 849 | ||
| Randomized Trials | 849 | ||
| Combination therapy to facilitate transition from intravenous/subcutaneous infused therapy to oral therapy | 851 | ||
| Combination therapy meta-analysis | 852 | ||
| Summary | 852 | ||
| References | 853 | ||
| Lung Transplantation and Atrial Septostomy in Pulmonary Arterial Hypertension | 857 | ||
| Key points | 857 | ||
| Introduction | 857 | ||
| Atrial septostomy | 858 | ||
| Lung and heart-lung transplantation | 859 | ||
| Waiting list outcomes in PAH | 859 | ||
| Outcomes after lung transplantation | 860 | ||
| Type of transplant procedure | 862 | ||
| Recommendations for timing of referral and listing for lung transplantation | 862 | ||
| Summary | 863 | ||
| References | 863 | ||
| Novel Medical Therapies for Pulmonary Arterial Hypertension | 867 | ||
| Key points | 867 | ||
| Introduction | 867 | ||
| New drugs in the final stages of drug development | 868 | ||
| The Endothelin Pathway | 868 | ||
| The Prostacyclin Pathway | 869 | ||
| Selexipag (ACT-293987) | 870 | ||
| The NO Pathway | 870 | ||
| Riociguat (BAY63-2521) | 870 | ||
| β-Blockers | 871 | ||
| New drugs less likely to reach clinical use | 871 | ||
| Imatinib (Gleevec) | 871 | ||
| Other TKIs | 872 | ||
| Serotonin | 872 | ||
| Vasoactive intestinal peptide | 873 | ||
| Statins | 873 | ||
| Cicletanine | 873 | ||
| New drugs in the early stages of development | 873 | ||
| Rho-Kinase Pathway | 873 | ||
| Dichloroacetate | 874 | ||
| Angiotension-Converting Enzyme 2 | 874 | ||
| Rituximab (Rituxan) | 874 | ||
| Other Potential Targets | 874 | ||
| Endothelial progenitor cells | 875 | ||
| Summary | 875 | ||
| References | 876 | ||
| Index | 881 |