This book provides the framework for a singular reference in the field of pulmonary
hypertension. Pulmonary vascular disease is a complex and heterogeneous condition
characterized by remodeling of distal pulmonary arterioles that increases pulmonary
vascular resistance to affect cardiopulmonary hemodynamic and right ventricular
function adversely, resulting in a clinical syndrome of diminished exercise
tolerance, shortness of breath, and heart failure-associated morbidity and mortality.
Owing to the availability of novel pulmonary circulation-selective pharmacotherapies
over the previous decade, the number of pulmonary hypertension patients eligible
for treatment has increased substantially. Despite this progress, under-awareness
persists within the practicing pulmonary, cardiovascular, and general internal
medicine communities. This is due, in part, to the complex array of molecular
mechanisms implicated in the pathobiology of PH, as well as cutting-edge discoveries
from translational scientific works that provide a new framework by which to
understand pulmonary vascular-right ventricular coupling.
Taken together, a key educational opportunity is exposed to bridge this knowledge gap through the synthesis of a contemporary text that emphasizes basic science, translational and clinical principles, and treatment strategies for understanding pulmonary hypertension.
Historical Perspective on the Classification and Nomenclature of Pulmonary Hypertension.- The Defining Characteristics of Pulmonary Arterial Hypertension.- Pulmonary Hypertension in Patients Without Pulmonary Arterial Hypertension.- Epidemiology of Pulmonary Hypertension: From Quaternary Referral Centre to the Community.- The Effects of Chronic Hypoxia on Inflammation and Pulmonary Vascular Function.- Genetics of Pulmonary Vascular Disease.- Novel Mechanisms of Disease: Network Biology and MicroRNA Signaling in Pulmonary Hypertension.- Pulmonary Hypertension as a Metabolic Disease.- Renin-Angiotensin-Aldosterone and other Neurohumoral Factors in the Pathogenesis of Pulmonary Hypertension.- Animal Models of Pulmonary Hypertension.- The Cardiopulmonary Hemodynamic Evaluation of Pulmonary Hypertension.- Advanced Imaging in Pulmonary Hypertension.- Assessing Disease State in the Pulmonary Vasculature in Clinical Practice and Research.- Biomarkers and other Methods for Assessing Patient Progress.- Pulmonary Circulatory - Right Ventricular Uncoupling: New Insights into Pulmonary Hypertension Pathophysiology.- Contemporary Pharmacotherapies Involving Nitric Oxide, Prostacyclin, and Endothelin Receptor Signaling Pathways.- Determining the Optimal Approach to Initiating Oral, Inhaled, and Intravenous Therapies in Clinical Practice: Sequential Goal-Directed Therapy is Best.- Counterpoint: Determining the optimal approach to initiating oral, inhaled, and intravenous therapies in clinical practice: Maximal upfront therapy is best.- Surgery, Devices, Transplantation and Other Interventional Options for the Treatment of Advanced Pulmonary Hypertension.- Patient Registries in Pulmonary Arterial Hypertension: the Role of Survival Equations and Risk Calculators.- Support Care for the Pulmonary Hypertension Patient.- Special Considerations for the Pulmonary Hypertension Patient.- The Future of Pulmonary Hypertension.