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This volume focuses on current evidence-based pharmacological treatments of various forms of pulmonary hypertension and provides a comprehensive review of the latest developments in this area. The first part of the book covers the definition, classification, pathophysiology, pathology, biomarkers and animal models of the disease, thus laying the conceptual basis for what follows. The middle section provides an overview of the established therapies, such as calcium channel blockers, prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors and inhaled nitric oxide. The last section explores novel pathways and emerging therapeutic approaches including soluble guanylate cyclase stimulators, Rho-kinase inhibitors, inhibitors of serotonin receptors and transporters, peptide growth factors, vasoactive peptides, modulators of redox equilibrium and cyclic nucleotide homeostasis, as well as immunosuppressive and anti-proliferative agents. Particular attention is given to the clinical applications of these experimental therapies, that are on the horizon. The book thus spans the continuum from basic science to clinical applications.

Diagnosing rare diseases can be challenging, and treating these conditions is complex because of their often quite specific needs and treatment options. To address this, the European Respiratory Society (ERS) has published Rare Diseases of the Respiratory System. Structured into thematic sections, the book covers: the identification of rare diseases of the respiratory system and their differential diagnosis; rare diseases of the lung interstitium; rare diseases of the airways or alveoli; and rare pulmonary vascular diseases. The Guest Editors and authors belong to and/or support the vision and mission of the European Reference Network for Rare Diseases of the Respiratory System (ERN-LUNG), which offers expert support to both patients and professionals. As such, this comprehensive book will prove an excellent resource for healthcare professionals, researchers and students interested in rare diseases of the respiratory system.

This Monograph aims to provide an in-depth overview of our current understanding of the various forms of pulmonary hypertension, their diagnosis and their treatment.

Written by internationally recognized experts, Pulmonary Hypertension bridges the gap between pulmonology and cardiology to provide clinicians in both disciplines with knowledge of the signs, symptoms, diagnosis, and pharmacologic and surgical treatments for pulmonary hypertension (PH). Through the use of clinical trials, this ground-breaking text