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European Heart Journal 2004 25(24):2243-2278; doi:10.1016/j.ehj.2004.09.014
Copyright © 2004 by the European Society of Cardiology.
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ESC Guidelines

Guidelines on diagnosis and treatment of pulmonary arterial hypertension

The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology

Task Force members, Nazzareno Galiè, (Chairperson) (Italy)1, Adam Torbicki, (Poland), Robyn Barst, (USA), Philippe Dartevelle, (France), Sheila Haworth, (UK), Tim Higenbottam, (UK), Horst Olschewski, (Germany), Andrew Peacock, (UK), Giuseppe Pietra, (Switzerland), Lewis J. Rubin, (USA) and Gerald Simonneau, (Co-Chairperson) (France)

The first 150 words of the full text of this article appear below.

ESC Committee for Practice Guidelines (CPG): Silvia G. Priori (Chairperson) (Italy), Maria Angeles Alonso Garcia (Spain), Jean-Jacques Blanc (France), Andrzej Budaj (Poland), Martin Cowie (UK), Veronica Dean (France), Jaap Deckers (The Netherlands), Enrique Fernandez Burgos (Spain), John Lekakis (Greece), Bertil Lindahl (Sweden), Gianfranco Mazzotta (Italy), Keith McGregor (France), João Morais (Portugal), Ali Oto (Turkey), Otto A. Smiseth (Norway)

Document reviewers: Gianfranco Mazzotta (CPG Review Coordinator) (Italy), Joan Albert Barbera (Spain), Simon Gibbs (UK), Marius Hoeper (Germany), Marc Humbert (France), Robert Naeije (Belgium), Joanna Pepke-Zaba (UK)

Preamble

Guidelines and Expert Consensus Documents aim to present all the relevant evidence on a particular issue in order to help physicians to weigh the benefits and risks of a particular diagnostic or therapeutic procedure. They should be helpful in everyday clinical decision-making.

A great number of Guidelines and Expert Consensus Documents have been issued in recent years by the European Society of Cardiology (ESC) and . . . [Full Text of this Article]

Introduction

Clinical classification of pulmonary hypertension

Idiopathic pulmonary arterial hypertension
Risk factors and associated conditions
Pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis
Classification of congenital systemic-to-pulmonary shunts
Pathology of pulmonary arterial hypertension

Pulmonary arteriopathy
Pulmonary occlusive venopathy (also called pulmonary veno-occlusive disease)
Pulmonary microvasculopathy (also called pulmonary capillary haemangiomatosis)
Pathogenesis of pulmonary arterial hypertension

Diagnostic strategy

Clinical suspicion of pulmonary hypertension
Detection of pulmonary hypertension
ECG
Chest radiograph
Transthoracic Doppler-echocardiography
Pulmonary hypertension clinical class identification
Pulmonary function tests and arterial blood gases
Ventilation and perfusion (V/Q) lung scan
High resolution CT of the lung
Contrast enhanced spiral CT of the lung, pulmonary angiography and magnetic resonance imaging
Pulmonary arterial hypertension evaluation (type, exercise capacity, haemodynamics)
Blood tests and immunology
Abdominal ultrasound scan
Exercise capacity
Haemodynamics
Lung biopsy
Assessment of severity

Clinical variables
Exercise capacity
Echocardiographic parameters
Haemodynamics
Blood tests
Treatment

Introduction to level of evidence and grade of recommendation
General measures
Pharmacological treatment
Oral anticoagulant treatment
Diuretics
Oxygen
Digitalis and dobutamine
Calcium-channel blockers
Synthetic prostacyclin and prostacyclin analogues
Endothelin-1 receptor antagonists
Type 5 phosphodiesterase inhibitors
Combination therapy
Interventional procedures
Balloon atrial septostomy
Lung transplantation
Treatment algorithm
Specific conditions

Paediatric pulmonary arterial hypertension
Pulmonary arterial hypertension associated to Eisenmenger syndrome
Porto-pulmonary hypertension
Pulmonary arterial hypertension associated to HIV infection
Pulmonary arterial hypertension associated to connective tissue diseases
Pulmonary veno-occlusive disease and pulmonary capillary haemangiomatosis
Appendix A: Abbreviations


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