Treatment of Pulmonary Hypertension

Book Preface

The definition of pulmonary hypertension (PH) is an elevated resting mean pulmonary artery pressure (mPAP) of greater than or equal to 25 mmHg, determined by right heart catheterisation [ 1 ]. It is a progressive and ultimately fatal disease without appropriate management. Many different diseases can be associated with this elevation in mPAP and therefore PH is a diverse clinical entity.

PH can be broadly categorised into those conditions that are associated with the histological features of plexogenic pulmonary arteriopathy (PPA [ 2 ]) and those which are not. Conditions associated with PPA are grouped under the umbrella term pulmonary arterial hypertension (PAH). In PAH the left atrial pressure is usually normal, and is measured as the pulmonary wedge pressure (PWP) during right heart catheterisation (PWP <15 mmHg). The difference between mPAP and PWP is then divided by cardiac output to calculate pulmonary vascular resistance (PVR), and this is significantly elevated in PAH. Examples of diseases in the group PAH include idiopathic, heritable and PAH in association with certain drugs or toxins, scleroderma, human immunodeficiency virus (HIV) infection or portal hypertension.

Many patients develop PH in association with cardiopulmonary diseases. PH is commonly associated with left heart disease (such as mitral valve disease), chronic lung diseases (such as sleep disordered breathing, lung parenchymal and airway diseases) and chronic pulmonary thromboemboli (termed chronic thromboembolic pulmonary hypertension (CTEPH)). These patients do not develop PPA for reasons that are unclear and have different pathophysiology, and therefore require different management strategies.

Contents
1 The Pathophysiology, Presentation and Diagnostic Investigation of Pulmonary Hypertension. . . .. . . 1
Jenny Bacon and Brendan Madden
2 Molecular Biological Aspects, Therapeutic Targets and New Treatment Strategies. . . . . . . . . . 37
Dongmin Shao, Laura Price, and Stephen John Wort
3 Current Treatment Strategies, Guidelines and New Therapies . . . . . . . . . . . . . 81
Adam Loveridge, Jenny Bacon, and Brendan Madden
4 The Role of Surgery in Pulmonary Hypertension . . . . . . . . . . . . . . . . . .. 147
Caroline Patterson
5 Putting It All Together . . . . . . . . . . . . . . . . . . . . . . . 171
Brendan Madden
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177