Pulmonary Hypertension Basic Science to Clinical Medicine 1st edition by Bradley Maron, Roham Zamanian, Aaron Waxman ISBN 3319235931 9783319235936

Part I: Overview and Classification of Pulmonary Hypertension

1. Historical Perspective on the Classification and Nomenclature of Pulmonary Hypertension

   • Introduction
   • Key Contributors with Keen Observations: The Evolution in Understanding Pulmonary Circulation
   • Introduction of Right Heart Catheterization
   • Measuring Hemodynamics in Health and Disease
   • Primary Pulmonary Hypertension and the Aminorex Epidemic
   • World Health Organization Meetings: A Timeline of Milestones
   • Nomenclature and Classification of Right Heart Failure
   • Conclusion

2. The Defining Characteristics of Pulmonary Arterial Hypertension (PAH)

   • Pathological Characteristics of Remodeling in the Pulmonary Vasculature
   • Inflammatory Pathology and Pathobiology of PAH
   • Role of Immune Response, Macrophages, and TGF-β Signaling
   • The Quasi Malignancy Paradigm of PAH
   • Hypoxia Signaling and Mitochondrial Dysfunction
   • Conclusion

3. Pulmonary Hypertension in Patients Without Pulmonary Arterial Hypertension

   • Pulmonary Hypertension Secondary to Left-Sided Heart Disease
   • Pulmonary Hypertension in Chronic Hypoxic Lung Diseases
   • The Role of Pulmonary Vasodilators in PH Management
   • Overlap Syndromes: Systemic Sclerosis, Sarcoidosis, and Other Conditions
   • Conclusion

4. Epidemiology of Pulmonary Hypertension

   • Prevalence and Incidence of Pulmonary Hypertension
   • Demographics and Comorbidities in PH
   • Subtypes and Associated Conditions in PH
   • Group 1 Pulmonary Arterial Hypertension (PAH) and Other WHO Groups
   • Summary and Conclusions

Part II: Basic Mechanisms of Disease

5. The Effects of Chronic Hypoxia on Inflammation and Pulmonary Vascular Function

   • Hypoxia-Induced Inflammatory Mechanisms and Vascular Remodeling
   • Role of Macrophages, Cytokines, and Epigenetics
   • Impact of Oxidative Stress in Chronic Hypoxia
   • Conclusion

6. Genetics of Pulmonary Vascular Disease

   • Genetics of Pulmonary Arterial Hypertension and Other Related Disorders
   • Impact of Genetics on Clinical Research and Medicine
   • Pediatric and Persistent Pulmonary Hypertension of the Newborn
   • Genetic Syndromes and Vascular Malformations
   • Conclusion

7. Novel Mechanisms of Disease: Network Biology and MicroRNA Signaling in Pulmonary Hypertension

   • Characteristics and Biogenesis of MicroRNAs
   • Role of MicroRNAs in PAH Pathogenesis and Potential Biomarkers
   • Future Directions in MicroRNA Research

8. Pulmonary Hypertension as a Metabolic Disease

   • Metabolic Dysregulation in PH: Glycolysis, TCA Cycle, and Fatty Acid Oxidation
   • Mitochondrial Dysfunction and its Impact on Right Ventricular Function
   • Therapeutic Metabolic Reprogramming in Pulmonary Hypertension
   • Summary and Future Directions

9. Renin-Angiotensin-Aldosterone and Other Neurohumoral Factors in the Pathogenesis of Pulmonary Hypertension

   • Adrenergic Signaling, Aldosterone, and the Renin-Angiotensin System in PH
   • The Role of Estrogen and Sex Hormones in PAH
   • Translational Insights for Therapeutic Targeting of Neurohumoral Pathways

Part III: Translational and Clinical Principles of Pulmonary Vascular Disease and Right Ventricular Dysfunction

10. Animal Models of Pulmonary Hypertension

    • Overview of Monocrotaline, SUGEN 5416, and Genetically Modified Models
    • Models for Specific Causes of Pulmonary Hypertension (e.g., Schistosomiasis, HIV)

11. The Cardiopulmonary Hemodynamic Evaluation of Pulmonary Hypertension

    • Hemodynamic Assessment Methods: Echocardiography, Invasive Catheterization, and Imaging
    • Systolic and Diastolic Pulmonary Artery Pressure
    • Pulmonary Vascular Resistance and Right Heart Function

12. Advanced Imaging in Pulmonary Hypertension

    • Role of Echocardiography, Chest Radiography, and CT Imaging in PH Diagnosis
    • Advances in MRI and PET for Pulmonary Hypertension

13. Assessing Disease State in the Pulmonary Vasculature in Clinical Practice and Research

    • Right Heart Catheterization and Exercise Testing
    • Imaging Techniques for Evaluating Right Heart and Pulmonary Circulation

14. Biomarkers and Other Methods for Assessing Patient Progress

    • Biomarkers for Endothelial Dysfunction, Inflammation, and Right Ventricular Failure
    • Clinical Tools for Monitoring Disease Progression

15. Pulmonary Circulatory – Right Ventricular Uncoupling: New Insights

    • Right Ventricular (RV)-Pulmonary Artery (PA) Coupling and its Clinical Implications
    • Insights from Experimental and Clinical Studies
    • Effects of Therapeutics on RV-PA Coupling

Part IV: Clinical Evaluation and Management of Pulmonary Hypertension

16. Contemporary Pharmacotherapies Involving Nitric Oxide, Prostacyclin, and Endothelin Receptor Signaling

    • Overview of Pharmacological Therapies: Prostacyclins, Endothelin Receptor Antagonists, and Nitric Oxide
    • Dosing, Side Effects, and Clinical Considerations

17. Determining the Optimal Approach to Initiating Therapies in Chronic Pulmonary Hypertension

    • Sequential Combination Therapy and Its Clinical Rationale
    • Limitations and Evidence Supporting Combination Therapy

18. Surgical and Interventional Approaches in Pulmonary Hypertension

    • Pulmonary Thromboendarterectomy, Atrial Septostomy, and Transplantation
    • Device-based Interventions and their Role in Advanced PH

19. Patient Registries in Pulmonary Arterial Hypertension

    • The Role of Patient Registries in Clinical Research and Prognosis Prediction
    • Survival Equations and Risk Scores in PAH Management

20. Supportive Care for the Pulmonary Hypertension Patient

    • Medication Education, Oxygen Therapy, and Palliative Care
    • Navigating Health Insurance and Support Networks

21. Special Considerations for the Pulmonary Hypertension Patient

    • Exercise, Pregnancy, and Perioperative Management in PH
    • Managing Pregnancy and Pain Control in Patients with PAH

22. The Future of Pulmonary Hypertension

    • Clinical Trial Design, Disease-Modifying Therapies, and Implantable Hemodynamic Monitors
    • New Paradigms for Therapeutic Strategies in Pulmonary Hypertension