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,
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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
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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|
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|
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|
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|
|
|
|
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|
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|
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|
|
|
|
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|
|
|
|
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|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
 |
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
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|
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|
|
|
|
|
|
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|
|
|
|
|
|
A. E Lammers, A. A Hislop, Y. Flynn, and S. G Haworth Epoprostenol treatment in children with severe pulmonary hypertension Heart, June 1, 2007; 93(6): 739 - 743. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|
|
|
 |
|
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|
|
|
|
|
|
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|
|
|
|
 |
|
 C.-W. Siu, X.-H. Zhang, C. Yung, A. W. C. Kung, C.-P. Lau, and H.-F. Tse Hemodynamic Changes in Hyperthyroidism-Related Pulmonary Hypertension: A Prospective Echocardiographic Study J. Clin. Endocrinol. Metab., May 1, 2007; 92(5): 1736 - 1742. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M D'Alto, C D Vizza, E Romeo, R Badagliacca, G Santoro, R Poscia, B Sarubbi, M Mancone, P Argiento, F Ferrante, et al. Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect Heart, May 1, 2007; 93(5): 621 - 625. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
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|
|
|
|
 |
|
 M.Y. Mok, P.L. Tsang, Y.M. Lam, Y. Lo, W.S. Wong, and C.S. Lau Bosentan use in systemic lupus erythematosus patients with pulmonary arterial hypertension Lupus, April 1, 2007; 16(4): 279 - 285. [Abstract] [PDF]
|
|
|
|
|
|
V. Tapson Atrial Septostomy: Why We Still Need It Chest, April 1, 2007; 131(4): 947 - 948. [Full Text] [PDF]
|
|
|
|
|
|
M. Kurzyna, M. Dabrowski, D. Bielecki, A. Fijalkowska, P. Pruszczyk, G. Opolski, J. Burakowski, M. Florczyk, W. Z. Tomkowski, L. Wawrzynska, et al. Atrial Septostomy in Treatment of End-Stage Right Heart Failure in Patients With Pulmonary Hypertension Chest, April 1, 2007; 131(4): 977 - 983. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. G. Blyth, B. A. Groenning, P. B. Mark, T. N. Martin, J. E. Foster, T. Steedman, J. J. Morton, H. J. Dargie, and A. J. Peacock NT-proBNP can be used to detect right ventricular systolic dysfunction in pulmonary hypertension Eur. Respir. J., April 1, 2007; 29(4): 737 - 744. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Hardziyenka, H. J. Reesink, B. J. Bouma, H.A.C.M. R. de Bruin-Bon, M. E. Campian, M. W.T. Tanck, R. B.A. van den Brink, J. J. Kloek, H. L. Tan, and P. Bresser A novel echocardiographic predictor of in-hospital mortality and mid-term haemodynamic improvement after pulmonary endarterectomy for chronic thrombo-embolic pulmonary hypertension Eur. Heart J., April 1, 2007; 28(7): 842 - 849. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. C. Mathai, R. E. Girgis, M. R. Fisher, H. C. Champion, T. Housten-Harris, A. Zaiman, and P. M. Hassoun Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension Eur. Respir. J., March 1, 2007; 29(3): 469 - 475. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Q. Ruan and S. F. Nagueh Clinical Application of Tissue Doppler Imaging in Patients With Idiopathic Pulmonary Hypertension Chest, February 1, 2007; 131(2): 395 - 401. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. P. McCann, C. T. Gan, A. M. Beek, H. W. M. Niessen, A. V. Noordegraaf, and A. C. van Rossum Extent of MRI Delayed Enhancement of Myocardial Mass Is Related to Right Ventricular Dysfunction in Pulmonary Artery Hypertension Am. J. Roentgenol., February 1, 2007; 188(2): 349 - 355. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. T Wittbrodt and A. Abubakar Sitaxsentan for Treatment of Pulmonary Hypertension Ann. Pharmacother., January 1, 2007; 41(1): 100 - 105. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. M. Hoeper, S. H. Lee, R. Voswinckel, M. Palazzini, X. Jais, A. Marinelli, R. J. Barst, H. A. Ghofrani, Z.-C. Jing, C. Opitz, et al. Complications of Right Heart Catheterization Procedures in Patients With Pulmonary Hypertension in Experienced Centers J. Am. Coll. Cardiol., December 19, 2006; 48(12): 2546 - 2552. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Provencher Long-term treprostinil in pulmonary arterial hypertension: is the glass half full or half empty? Eur. Respir. J., December 1, 2006; 28(6): 1073 - 1075. [Full Text] [PDF]
|
|
|
|
|
|
C. T. Gan, G. P. McCann, J. T. Marcus, S. A. van Wolferen, J. W. Twisk, A. Boonstra, P. E. Postmus, and A. Vonk-Noordegraaf NT-proBNP reflects right ventricular structure and function in pulmonary hypertension Eur. Respir. J., December 1, 2006; 28(6): 1190 - 1194. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 O. Distler, F. Behrens, D. Huscher, I. Foeldvari, A. Zink, P. Nash, C. P. Denton, M. Humbert, M. Matucci-Cerinic, J. Seibold, et al. Need for improved outcome measures in pulmonary arterial hypertension related to systemic sclerosis Rheumatology, December 1, 2006; 45(12): 1455 - 1457. [Full Text] [PDF]
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