Table of Contents

PHYSIOLOGY AND PATHOPHYSIOLOGY OF PULMONARY CIRCULATION AND RIGHT HEART

Evolution of the Pulmonary Circulation and the Right Heart
John B. West

Pulmonary Vascular Function
Robert Naeije

Gas Exchange
J.M.B. Hughes

Right Ventricular Function
Ryan J. Tedford

Heart-Lung Interactions
Xavier Repessé, Cyril Charron, and Antoine Vieillard-Baron

PATHOBIOLOGY OF PULMONARY VASCULAR DISEASE AND RIGHT VENTRICULAR FAILURE

Pathology of Pulmonary Vascular Diseases
Peter Dorfmüller

Pathobiology of Pulmonary Hypertension
Mark L. Ormiston and Nicholas W. Morrell

Pathobiology of Right Ventricular Failure
Anna R. Hemnes and Evan L. Brittain

CLINICAL ASSESSMENT OF PULMONARY HYPERTENSION

Clinical Features
Andrew J. Peacock and A. Colin Church

Echocardiography
Lawrence Rudski and Jonathan Afilalo

Imaging and Pulmonary Hypertension: Chest Radiography, Ventilation-Perfusion Scintigraphy, and Computed Tomography
Michael Sproule

Imaging: Emerging Modalities (Magnetic Resonance and Nuclear Imaging)
A. Vonk Noordegraaf and O.A. Spruijt

Exercise Testing in Pulmonary Hypertension
Daniel Dumitrescu and Ronald J. Oudiz

Clinical Assessment of PH: Biomarkers
Adam Torbicki, Anna Fijałkowska, and Marcin Kurzyna

Cardiac Catheterization
Gabor Kovacs and Horst Olschewski

Classification of Pulmonary Hypertension
Lewis J. Rubin

GROUP 1: PULMONARY ARTERIAL HYPERTENSION

Idiopathic Pulmonary Arterial Hypertension
Lewis J. Rubin

Heritable Pulmonary Hypertension
James E. Loyd, John H. Newman, and Eric D. Austin

Pulmonary Arterial Hypertension Associated with Drugs and Toxins
Olivier Sitbon, Caroline O’Connell, Laurent Savale, David Montani, Marc Humbert, and Gerald Simonneau

Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases
Todd M. Kolb and Paul M. Hassoun

Portopulmonary Hypertension
Rodrigo Cartin-Ceba and Michael J. Krowka

Pulmonary Arterial Hypertension Associated with HIV Infection
Rudolf Speich

Pulmonary Hypertension Related to Congenital Systemic-to-Pulmonary Shunts
Michele D’Alto and Konstantinos Dimopoulos

Schistosomiasis
Francisca Gavilanes, Caio Julio Cesar Fernandes, Carlos Jardim, and Rogerio Souza

Pulmonary Hypertension in Pregnancy
Xavier Jaïs, Laura Claire Price, David Montani, Laurent Savale, Olivier Sitbon, Gérald Simonneau, Karen M. Olsson, and Marc Humbert

Pulmonary Venoocclusive Disease and Pulmonary Capillary Hemangiomatosis
Lewis J. Rubin

Pulmonary Arterial Hypertension Registries
Edmund M.T. Lau and Marc Humbert

DRUG AND NONDRUG TREATMENT OF GROUP 1 PULMONARY ARTERIAL HYPERTENSION

Conventional Medical Therapies: Supportive Therapies and General Clinical Care Measures
Mardi Gomberg-Maitland

Calcium Channel Blockers in the Treatment of Pulmonary Arterial Hypertension
Jonathan D. Rich and Stuart Rich

Prostacyclins
Vallerie V. McLaughlin and Harold I. Palevsky

Endothelin Receptor Antagonists
Daniel Fox, Steven Pugliese, Jeffrey Robinson, Brian Graham, Edward C. Dempsey, Todd M. Bull, and David B. Badesch

Phosphodiesterase Type-5 Inhibitors
Mark Toshner and Joanna Pepke-Zaba

Soluble Guanylate Cyclase Stimulators and Activators
Hossein-Ardeschir Ghofrani, Manuel Jonas Richter, Jan Grimminger, Hennig Gall, Werner Seeger, and Friedrich Grimminger

Rehabilitation
Ekkehard Grünig and Nicola Benjamin

Combination Therapy for Pulmonary Arterial Hypertension
Marius M. Hoeper

Atrial Septostomy and Potts Procedure
Julio Sandoval and Jorge Gaspar

Lung Transplantation for Pulmonary Arterial Hypertension Including Bridging to Transplantation
Paul A. Corris

Future of Pulmonary Arterial Hypertension Therapy
Astrid Weiss, Tatyana Novoyatleva, Baktybek Kojonazarov, and Ralph Theo Schermuly

GROUP 2: PULMONARY HYPERTENSION ASSOCIATED WITH LEFT HEART CONDITIONS

World Health Organization Pulmonary Hypertension Group 2: Pulmonary Hypertension due to Left Heart Disease
Gregory D. Lewis

Diagnosis and Clinical Relevance of Pulmonary Hypertension Secondary to Mitral or Aortic Valve Diseases or Congenital Left-Sided Cardiac Abnormalities
Patrizio Lancellotti, Christine Selton-Suty, and Damien Voilliot

Treatment of Pulmonary Hypertension in Left Heart Disease
Marco Guazzi

GROUP 3: PULMONARY HYPERTENSION ASSOCIATED WITH HYPOXIA, HYPOVENTILATION, AND LUNG DISEASE

Chronic High-Altitude Exposure
Dante Penaloza

Hypoventilation and Sleep Apnea Syndromes
Ari Chaouat, Emmanuel Gomez, Magalie Mercy, Anne Guillaumot, and François Chabot

Chronic Obstructive Pulmonary Disease (COPD)
Joan Albert Barbera and Isabel Blanco

Interstitial Lung Disease and Pulmonary Hypertension
Steven D. Nathan

Combined Pulmonary Fibrosis and Emphysema Syndrome
Vincent Cottin

GROUP 4: CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION

Diagnosis of Chronic Thromboembolic Pulmonary Hypertension
Irene M. Lang, Daniel Dalos, and Christian Gerges

Medical Therapy for Chronic Thromboembolic Pulmonary Hypertension
Nick H. Kim

Balloon Pulmonary Angioplasty
Hiromi Matsubara and Aiko Ogawa

Pulmonary Thromboendarterectomy
Michael M. Madani

PULMONARY HYPERTENSION WITH UNCLEAR AND/OR MULTIFACTORIAL MECHANISMS

"Group 5" Pulmonary Hypertension with Unclear and/or Multifactorial Mechanisms
Andrew J. Peacock

Pulmonary Hypertension in Sickle Cell Disease and Thalassemia
Claudia R. Morris and Mark T. Gladwin

ACUTE PULMONARY EMBOLISM

Diagnosis of Acute Pulmonary Thromboembolism
Arnaud Perrier and Marc Righini

Treatment of Acute Pulmonary Embolism
Stavros V. Konstantinides

PEDIATRIC PULMONARY HYPERTENSION

Pulmonary Hypertension in Children: A Global Perspective
R.M.F. Berger

Idiopathic Pulmonary Arterial Hypertension in Children
Dunbar Ivy

Persistent Pulmonary Hypertension of the Newborn: Pathophysiology and Treatment
Erica Mandell, Steven H. Abman, and Robin H. Steinhorn

PULMONARY CIRCULATION IN CRITICAL CARE

Acute Lung Injury and Acute Respiratory Distress Syndrome
Laura Claire Price and S. John Wort

Severe Right Ventricular Failure
Rebecca Vanderpool and Marc A. Simon

PULMONARY CIRCULATION IN ENVIRONMENTAL STRESS

Exercise
André La Gerche

Pulmonary Circulation at High Altitude
Erik R. Swenson

PULMONARY ARTERIO-VENOUS MALFORMATIONS AND SHUNTS

Pulmonary Circulation After Palliative Cavopulmonary Connections
Mathieu Vergnat and Elie Fadel

The Hepatopulmonary Syndrome
Michael B. Fallon and Moises I. Nevah Rubin

Pulmonary Arteriovenous Malformations
Claire L. Shovlin

PULMONARY VASCULAR TUMORS AND PULMONARY VASCULITIS

Pulmonary Vascular Tumors
Demosthenes G. Papamatheakis and Kim M. Kerr

Pulmonary Vasculitis
Cassandra Batzlaff and Karina Keogh