Haemodynamic definitions and updated clinical classification of pulmonary hypertension.
Gérald Simonneau,Gérald Simonneau,David Montani,David Montani,David S. Celermajer,Christopher P. Denton,Michael A. Gatzoulis,Michael J. Krowka,Paul G. Williams,Rogério Souza +9 more
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TLDR
The 6th WSPH Task Force proposed to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg, and included in group 1 of a subgroup “pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers”.Abstract:
Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3.3 mmHg. Two standard deviations above this mean value would suggest mPAP >20 mmHg as above the upper limit of normal (above the 97.5th percentile). This definition is no longer arbitrary, but based on a scientific approach. However, this abnormal elevation of mPAP is not sufficient to define pulmonary vascular disease as it can be due to an increase in cardiac output or pulmonary arterial wedge pressure. Thus, this 6th WSPH Task Force proposes to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg. Prospective trials are required to determine whether this PH population might benefit from specific management. Regarding clinical classification, the main Task Force changes were the inclusion in group 1 of a subgroup “pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers”, due to the specific prognostic and management of these patients, and a subgroup “PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement”, due to evidence suggesting a continuum between arterial, capillary and vein involvement in PAH.read more
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References
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2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).
Nazzareno Galiè,Marc Humbert,Jean-Luc Vachiery,Simon Gibbs,Irene Lang,Adam Torbicki,Gérald Simonneau,Andrew Peacock,Anton Vonk Noordegraaf,Maurice Beghetti,Ardeschir Ghofrani,Miguel Angel Gomez Sanchez,Georg Hansmann,Walter Klepetko,Patrizio Lancellotti,Marco Matucci,Theresa McDonagh,Luc Pierard,Pedro T. Trindade,Maurizio Zompatori,Marius M. Hoeper +20 more
TL;DR: In this paper, the authors defined the following terms: ALAT, alanine aminotransferase, ASAT, aspartate AMINOTE, and APAH, associated pulmonary arterial hypertension.
Journal ArticleDOI
Updated clinical classification of pulmonary hypertension.
Gérald Simonneau,Michael A. Gatzoulis,Michael A. Gatzoulis,Ian Adatia,Ian Adatia,David S. Celermajer,David S. Celermajer,Christopher P. Denton,Ardeschir Ghofrani,Miguel Angel Gomez Sanchez,R. Krishna Kumar,Michael J. Landzberg,Roberto F. Machado,Horst Olschewski,Ivan M. Robbins,Rogiero Souza +15 more
TL;DR: In this paper, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches, and the main change was to withdraw persistent pulmonary hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups.
Journal ArticleDOI
Clinical classification of pulmonary hypertension
Gérald Simonneau,Nazzareno Galiè,Lewis J. Rubin,David Langleben,Werner Seeger,Guido Domenighetti,Simon Gibbs,Didier Lebrec,Rudolf Speich,Maurice Beghetti,Stuart Rich,Alfred P. Fishman +11 more
TL;DR: The growth of understanding of the pulmonary hypertensive diseases that has enabled the development of a clinical classification oriented towards prevention and treatment is traced.
Journal ArticleDOI
The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension.
TL;DR: This study suggests that high doses of calcium-channel blockers in patients with primary pulmonary hypertension who respond with reductions in pulmonary-artery pressure and pulmonary vascular resistance may improve survival over a five-year period.
Journal ArticleDOI
Guidelines for the diagnosis and treatment of pulmonary hypertension
Marius M. Hoeper,Marc Humbert,Adam Torbicki,Jean-Luc Vachiery,Joan Albert Barberà,Maurice Beghetti,Paul A. Corris,Sean Gaine,J. Simon R. Gibbs,Miguel Ángel Gómez-Sánchez,Guillaume Jondeau,Walter Klepetko,Andrew J. Peacock,Lewis J. Rubin,Michael J. Zellweger,Gérald Simonneau,Angelo Auricchio,Jeroen J. Bax,Claudio Ceconi,Veronica Dean,Gerasimos Filippatos,Christian Funck-Brentano,Richard J. Hobbs,Peter Kearney,Theresa McDonagh,Keith McGregor,Bogdan A. Popescu,Zeljko Reiner,Udo Sechtem,Michal Tendera,Panos E. Vardas,Petr Widimsky,Felicita Andreotti,Michael Aschermann,Riccardo Asteggiano,Ray Benza,Rolf M. F. Berger,Damien Bonnet,Marion Delcroix,Luke Howard,Anastasia Kitsiou,Irene Lang,Aldo P. Maggioni,Jens Erik Nielsen-Kudsk,Myung H. Park,Pasquale Perrone-Filardi,Maria Teresa Subirana Domenech,Anton Vonk-Noordegraaf,José Luis Zamorano +48 more
TL;DR: Guidelines and Expert Consensus Documents summarise and evaluate all currently available evidence on a particular issue with the aim to assist physicians in selecting the best management strategies for a typical patient, suffering from a given condition, taking into account the impact on outcome, as well as the risk/benefit ratio of particular diagnostic or therapeutic means.
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