Open Access Medical Books

TEXTBOOK : PULMONARY HYPERTENSION – FROM BENCH RESEARCH TO CLINICAL CHALLENGES

PULMONARY HYPERTENSION

Edited by Roxana Sulica and Ioana Preston .

Open Access .
388 pages .

The lung has a unique vascular structure and function; it has low pressure, low resistance circulation with a highly compliant system which accommodates the same amount of flow as the systemic circulation. In addition, pulmonary and systemic vasculatures have divergent responses to various stimuli. For example, pulmonary arteries constrict in the setting of hypoxia, while systemic circulation dilates. This is due to distinctive developmental characteristics, anatomic and histological structure, as well as physiological properties. These properties stem from a particular array of molecular and cellular mediators which favor vasodilation and maintenance of thinwalled and pliable pulmonary resistance vessels. 
Pulmonary hypertension is characterized by a mediator imbalance with a predominance of vasoconstriction and cell proliferation involving all layers of the vessel. The end result is an increase in pulmonary vascular resistance, increased workload of the right ventricle, and right ventricular hypertrophy to maintain an adequate flow. Subsequently, right ventricular dilatation ensues the signs and symptoms of right heart failure occurence. Therefore, while the origin of the anatomical disturbance is at the level of the pulmonary arteries resistance, the end result is right heart failure.
While there are still gaps in understanding pulmonary vasculature, tremendous progress has been made in understanding its functionality, its adaptation to hypoxia, the effects of increased pulmonary vascular resistance on the right ventricular function, and the molecular pathways affected in this process. As a result, there are currently seven therapies for pulmonary arterial hypertension and the field is rapidly moving forward, with several novel molecular targets under development. Therefore, a textbook addressing developmental, cellular and clinical findings about pulmonary vascular disorders is relevant and much needed.
The textbook “Pulmonary Hypertension - From Bench Research to Clinical Challenges” addresses the following topics: structure and function of the normal pulmonary vasculature; disregulated cellular pathways seen in experimental and human pulmonary hypertension; clinical aspects of pulmonary hypertension in general; presentation of several specific forms of pulmonary hypertension, and management of pulmonary hypertension in special circumstances. Therefore, the textbook should be of interest to basic and clinical researchers with particular interest in pulmonary vascular diseases; clinicians and trainees (clinical and research fellows, residents and students).
This book is unique in that it combines pulmonary and cardiac physiology and pathophysiology with clinical aspects of the disease. The first two sections which comprise seven chapters include the basic knowledge and the recent discoveries related to structure and cellular function of the pulmonary vasculature. The chapters also describe disregulated pathways known to be affected in pulmonary hypertension.
A special section deals with the effects of hypoxia on the pulmonary vasculature and the myocardium. The remaining three sections of the book comprise ten chapters and introduce the methods of evaluating pulmonary hypertension to the reader. The chapters present several forms of pulmonary hypertension which are particularly challenging in clinical practice (such as pulmonary arterial hypertension associated with systemic sclerosis), and lastly, they address special considerations regarding management of pulmonary hypertension in certain clinical scenarios such as pulmonary hypertension in the critically ill.
The textbook is written by international scientists and physicians with expertise in pulmonary vascular diseases. Many of them are active in preclinical, translational and clinical research involving the pulmonary vasculature, as well as in treating patients with various forms and severities of pulmonary hypertension.
We hope that the textbook will enhance the scientists' knowledge about the complexities of the pulmonary vasculature, stimulate trainees to dedicate part of their future clinical work to understanding and treating patients with pulmonary hypertension, and that it will increase the clinicians' awareness to the importance of correct and early diagnosis and adequate treatment.

Dr. Roxana Sulica,
Director of the Beth Israel Pulmonary Hypertension Program,
Assistant Professor of Medicine,
Albert Einstein College of Medicine, USA
Ioana R. Preston, MD
Assistant Professor of Medicine
Tufts University School of Medicine
Co-Director, Pulmonary Hypertension Center
Pulmonary, Critical Care and Sleep Division
Tufts Medical Center, Boston, MA, USA
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CONTENTS : 

Part 1 of the Textbook : Pulmonary Vascular Function and Dysfunction .


 1 Pulmonary Hypertension: Endothelial Cell Function 3 Rajamma Mathew

 2 Integrin-Mediated Endothelial Cell Adhesion and Activation of c-Src, EGFR and ErbB2 are Required for Endothelial-Mesenchymal Transition 25 Enrique Arciniegas, Luz Marina Carrillo, Héctor Rojas and José Cardier

 3 Interplay Between Serotonin Transporter Signaling and Voltage-Gated Potassium Channel (Kv) 1.5 Expression 49 Christophe Guignabert

 4 Deregulation of BMP Signaling in the Pathogenesis of Pulmonary Hypertension 67 Miriam de Boeck and Peter ten Dijke

 5 3 and 6 CYP450 Eicosanoid Derivatives: Key Lipid Mediators in the Regulation of Pulmonary Hypertension 83 Caroline Morin, Samuel Fortin, Christelle Guibert and Éric Rousseau

Part 2 of the Textbook : Hypoxia and Its Effects on Pulmonary Vasculature and Heart .


 6 Hypoxic Pulmonary Arterial Hypertension in the Chicken Model 111 Aureliano Hernández and Martha de Sandino

 7 Inadequate Myocardial Oxygen Supply/Demand in Experimental Pulmonary Hypertension 151 B. J. van Beek-Harmsen, H. M. Feenstra and W. J. van der Laarse

Part 3 of the Textbook : Clinical Evaluation and Diagnostic Approaches for Pulmonary Hypertension .


 8 Assessment of Structural and Functional Pulmonary Vascular Disease in Patients with PAH 169 Juan Grignola, Enric Domingo, Rio Aguilar-Torres and Antonio Roman

 9 Dyspnea in Pulmonary Arterial Hypertension 191 Dimitar Sajkov, Karen Latimer and Nikolai Petrovsky

 10 Echocardiography in Pulmonary Hypertension 209 Chin-Chang Cheng and Chien-Wei Hsu

Part 4 of the Textbook : Several Clinical Forms of Pulmonary Hypertension .


 11 Pulmonary Hypertension in Systemic Sclerosis 221 Muhammad Ishaq Ghauri, Jibran Sualeh Muhammad and Kamran Hameed

 12 Clinical Syndromes and Associations with Persistent Pulmonary Hypertension of the Newborn 231 Jae H. Kim and Anup Katheria

 13 Sarcoidosis Associated Pulmonary Hypertension 253 Veronica Palmero, Phillip Factor and Roxana Sulica

 14 Pulmonary Hypertension in Patients with Chronic Kidney Disease 263 Alessandro Domenici, Remo Luciani, Francesco Principe, Francesco Paneni, Giuseppino Massimo Ciavarella and Luciano De Biase

Part 5 of the Textbook : Special Considerations in Evaluation and Management of Pulmonary Hypertension .


 15 Perioperative Management of Pulmonary Hypertension 275 Philip L. Kalarickal, Sabrina T. Bent, Michael J. Yarborough, Kavitha A. Mathew and Charles Fox

 16 Pregnancy and Pulmonary Arterial Hypertension 289 Jean M. Elwing and Ralph J. Panos

 17 Pulmonary Hypertension in the Critically Ill 305 Michelle S. Chew, Anders Åneman, John F. Fraser and Anthony S. McLean 




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