Pulmonary Hypertension: Controversial and Emerging Topics

Contents
Contributors
Chapter 1: Exercise Pulmonary Hypertension
Introduction
Normal Resting and Exercise Pulmonary Hemodynamics
What Is the Normal Pulmonary Vascular Response to Exercise?
Exercise Pulmonary Hypertension
What Are the Hemodynamic Criteria to Define ePH?
Should Age Be Considered in the Definition of ePH?
Is There Any Benefit to Performing Confrontational Exercise Testing in Patients with an mPAP 21–24 mmHg?
What About the Wedge?
The Clinical Impact of ePH
Exercise Pulmonary Hypertension, Who Cares?
Is There Evidence of Pulmonary Vasculature Pathology in ePH?
Is There Evidence of Molecular Alterations in ePH That May Drive Disease?
Unresolved Questions and Controversies Regarding Exercise Hemodynamics to Define ePH
Is ePH a Distinct Clinical Entity?
How Should the Cardiac Output Be Measured to Define ePH?
What Do Novel Hemodynamic Measures Add to Our Understanding of ePH?
Alpha
Can ePH Be Predicted?
Can ePH Be Treated?
Methods to Assess Invasive Exercise Hemodynamics
What Methodologies Are Available to Assess Exercise Pulmonary Hypertension?
Conclusion
References
Chapter 2: Advanced Right Ventricular Assessment: Pulmonary Artery Compliance and RV-PA Coupling
Introduction
The Pulmonary Vascular System as Right Ventricular Load: Resistance vs. Compliance
Anatomy
Components of Right Ventricular Afterload
Relationship Between Resistance and Compliance
Effect of Pulmonary Artery Wedge Pressure on the Resistance-Compliance Relationship
Right Ventricular Function: Determinants, Assessment, and Pathophysiology
Determinants of Right Ventricular Function
Heart Rate and Rhythm
Preload, Afterload, and Interventricular Dependence
Contractility
Pathophysiology of Right Ventricular Dysfunction and Failure
Assessment of Right Ventricular Function
Ventricular-Vascular Coupling: The Right Ventricular-Pulmonary Arterial Unit
Pressure-Volume Loops of the Normal Left Ventricle
Effects of Ventricular Determinants on Pressure-Volume Loops of the Left Ventricle
Pressure-Volume Loops of the Right Ventricle
The Concept of Ventricular-Vascular Coupling
Clinical Applications of Ventricular-Vascular Coupling
Noninvasive Methods to Estimate Ventricular-Vascular Coupling
Diastolic Function of the Right Ventricle
Diastolic Stiffness in Patients with Pulmonary Hypertension
Glossary
References
Chapter 3: Beyond Scleroderma: Pulmonary Arterial Hypertension in Patients with Other Connective Tissue Diseases
Introduction
Epidemiology of CTD-PAH
Scleroderma
Mixed Connective Tissue Disease
Other Connective Tissue Diseases
Screening and Early Detection of CTD-PAH
Scleroderma Spectrum Diseases
SLE-PAH
Other CTDs
Treatment of CTD-PAH
Selexipag
Riociguat
Immunosuppression
Conclusion and Future Directions
Bibliography
Chapter 4: Isolated Postcapillary and Combined Pre- and Postcapillary Pulmonary Hypertension
Introduction
Do We Need Invasive Hemodynamics to Diagnose Postcapillary PH and to Differentiate Pulmonary Hypertension Due to Left Heart Disease (PH-LHD) Subsets With or Without Pulmonary Vascular Disease or Can We Substitute Right Heart Catheterization with Al
Hemodynamic Variables to Dissect Pulmonary Hypertension Due to Left Heart Disease (PH-LHD) Subsets: With or Without Pulmonary Vascular Disease (Table 4.1)
Definition and Classification of PH-LHD
Isolated Postcapillary and Combined Pre- and Postcapillary Pulmonary Hypertension: Are They Clinical Phenotypes?
Do We Need to Differentiate Isolated Postcapillary (Ipc-PH) from Combined Pre- and Postcapillary Pulmonary Hypertension (Cpc-PH)?
Conclusion
References
Chapter 5: Current Approach to Chronic Thromboembolic Disease Without Pulmonary Hypertension
Background
Pathogenesis and Epidemiology
Pathophysiology
Clinical Presentation
Diagnostic Evaluation
Treatment
Conclusion
References
Chapter 6: Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis
Introduction
Pathology
Clinical Classification
Epidemiology
Genetics
Nongenetic Risk Factors
Clinical Features and Diagnosis
Presenting Signs and Symptoms
Echocardiography and Hemodynamics
Radiology
Pulmonary Function Studies
Bronchoalveolar Lavage
Response to Therapy
Noninvasive Diagnosis of PVOD/PCH
Prognosis
Management
Lung Transplantation
Supportive Care
PAH-Specific Therapy
Immunosuppression
Other Therapies
Conclusion
References
Chapter 7: Controversies in the Management of Pulmonary Hypertension in the Setting of Lung Disease
Introduction
Pathogenesis
Diagnosis
What to Do with PH in the Context of Lung Disease Once It Is Detected?
Treat PH Complicating Lung Disease: Yes!
Treat PH Complicating Lung Disease: No!
Treat PH Complicating Lung Disease: Taking the Middle Road
Where to from Here for CLD-PH?
What to Do Now with CLD-PH?
Glossary
References
Chapter 8: Pulmonary Hypertension in Sickle Cell Disease: Current Controversies and Clinical Practices
Introduction
How Should PH of SCD Be Classified?
Diagnosis of PH in SCD
When Should One Perform a Right Heart Catheterization in Patients with SCD?
How Does One Diagnose PH of SCD Hemodynamically?
Which Investigations Should Be Performed in Patients with RHC-Confirmed PH?
How Should One Treat PH of SCD?
Which Patients with PH of SCD Should Be Treated with Anticoagulation?
Do Patients with PH of SCD Respond to PAH Therapy?
Conclusion
References
Chapter 9: Sarcoidosis-Associated Pulmonary Hypertension
Introduction
Pathophysiology
Diagnosis
Prevalence
History and Physical Exam
Pulmonary Function Testing
Serum Biomarkers
Six-Minute Walk Test
Echocardiography
Right Heart Catheterization
Treatment
Outcomes
Future Directions/Prognosis
References
Chapter 10: Parenteral Prostacyclin Use in Pulmonary Arterial Hypertension
Physiologic Effects of Prostacyclin
The Parenteral Prostacyclins
Epoprostenol
Practical Use and Side Effects
Treprostinil
Practical Use and Side Effects
Controversies in Prostacyclin Therapeutic Trials
Endpoints in Prostacyclin Trials
Study Duration
Patient Selection and Inclusion
WHO Group 1
Idiopathic and Heritable PAH
Connective Tissue Disease-Associated PAH
Congenital Heart Disease
Portopulmonary Hypertension
Drug and Toxin Associated
Human Immunodeficiency Virus (HIV)
Pulmonary Veno-occlusive Disease (PVOD)
Schistosomiasis
WHO Group 4
Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
WHO Group 5
Sarcoidosis
Implementation into Clinical Practice
Timing of Parenteral Therapy
Patient-Related Features
Psychosocial Components to Parenteral Therapy
Summary
References
Chapter 11: Pulmonary Hypertension in Chronic Kidney Disease and End-Stage Renal Disease
Introduction, Definitions, and Terminology
Epidemiology of Pulmonary Hypertension in CKD and ESRD Populations
Clinical Significance of PHTN and Kidney Disease Together
Etiology of Pulmonary HTN in CKD and ESRD
A) Comorbidities Connecting PH and CKD-ESRD (“Explained PH”)
B) Possible Causes of the “Unexplained” PH
Screening and Diagnosis
Treatment
Conclusion
Bibliography
Chapter 12: Gender and Race Disparities in Pulmonary Hypertension Diagnosis and Treatment
Introduction
Race/Ethnicity and PAH
Socioeconomic Status and PAH
Gender, Age, and PAH
Genetic Contributors to Healthcare Disparities in PAH
Future Directions in Healthcare Disparities in PAH
References
Index