The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension
Nils Nickel,Heiko Golpon,Mark Greer,Lars Knudsen,Karen M. Olsson,Volker Westerkamp,Tobias Welte,Marius M. Hoeper +7 more
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TLDR
During follow-up, changes in World Health Organization functional class, cardiac index, Sv,O2 and NT-proBNP proved significant predictors of outcome and when assigned to prognostic groups, improvements as well as deteriorations in these parameters after initiation of PAH-targeted therapy had a strong impact on survival.Abstract:
Current guidelines for the treatment of patients with idiopathic pulmonary arterial hypertension (IPAH) recommend basing therapeutic decision-making on haemodynamic, functional and biochemical variables. Most of these parameters have been evaluated as risk predictors at the time of diagnosis. The aim of the present study was to assess the prognostic impact of changes in these parameters after initiation of targeted therapy. A cohort of 109 patients with IPAH who had undergone haemodynamic, functional and biochemical assessments at baseline and 3-12 months after initiation of pulmonary arterial hypertension (PAH)-targeted therapy, were followed for a median 38 months in order to determine predictors of mortality at baseline and during the course of their disease. Within the observation period, 53 (48.6%) patients died and four (3.7%) underwent lung transplantation. Kaplan-Meier estimates for transplantation-free survival were 92%, 67%, and 51% at 1, 3, and 5 yrs, respectively. Among baseline variables, 6-min walk distance, right atrial pressure, cardiac index, mixed-venous oxygen saturation (S(v,O(2))) and N-terminal-pro brain natriuretic peptide (NT-proBNP) were independent predictors of survival. During follow-up, changes in World Health Organization functional class, cardiac index, S(v,O(2)) and NT-proBNP proved significant predictors of outcome. When assigned to prognostic groups, improvements as well as deteriorations in these parameters after initiation of PAH-targeted therapy had a strong impact on survival. Measurements obtained at follow-up had a higher predictive value than variables obtained at baseline. Changes in established predictors of outcome during the course of the disease provide important prognostic information in patients with IPAH.read more
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Journal ArticleDOI
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
Special article2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension
Nazzareno Galiè,Marc Humbert,Jean-Luc Vachiery,Simon Gibbs,Irene Lang,Adam Torbicki,Gérald Simonneau,Andrew J. Peacock,Anton Vonk Noordegraaf,Maurice Beghetti,Ardeschir Ghofrani,Miguel Angel Gomez Sanchez,Georg Hansmann,Walter Klepetko,Patrizio Lancellotti,Marco Matucci,Theresa McDonagh,Luc Pierard,Marius M. Hoeper +18 more
TL;DR: This article is being published concurrently in the European Heart Journal and the European Respiratory Journal and is identical except for minor stylistic and spelling differences in keeping with each journal’s style.
Journal ArticleDOI
[2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension].
Nazzareno Galiè,Marc Humbert,Jean-Luc Vachiery,Simon Gibbs,Irene Lang,Adam Torbicki,Gérald Simonneau,Andrew J. 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: 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) is constituted.
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.
Journal ArticleDOI
Definitions and diagnosis of pulmonary hypertension.
Marius M. Hoeper,Harm Jan Bogaard,Robin Condliffe,Robert P. Frantz,Dinesh Khanna,Marcin Kurzyna,David Langleben,Alessandra Manes,Toru Satoh,Fernando Torres,Martin R. Wilkins,David B. Badesch +11 more
TL;DR: Early diagnosis of PAH remains difficult, and screening programs in asymptomatic patients are feasible only in high-risk populations, particularly in patients with systemic sclerosis, for whom recent data suggest that a combination of clinical assessment and pulmonary function testing has a higher predictive value than echocardiography alone.
References
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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
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Journal ArticleDOI
Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT)
Nazzareno Galiè,Marius M. Hoeper,Marc Humbert,Adam Torbicki,Jean-Luc Vachiery,J.A. Barberà,Maurice Beghetti,Paul A. Corris,Sean Gaine,J S R Gibbs,Miguel Ángel Gómez-Sánchez,Guillaume Jondeau,Walter Klepetko,Christian Opitz,Andrew J. Peacock,LJ Rubin,Michael J. Zellweger,Gérald Simonneau +17 more
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Journal ArticleDOI
Survival in Patients with Primary Pulmonary Hypertension: Results from a National Prospective Registry
Gilbert E. D'Alonzo,Robyn J. Barst,Stephen M. Ayres,Edward H. Bergofsky,Bruce H. Brundage,Katherine M. Detre,Alfred P. Fishman,Roberta M. Goldring,Berton M. Groves,Janet T. Kernis,Paul S. Levy,Giuseppe G. Pietra,Lynne Reid,John T. Reeves,Stuart Rich,Carol E. Vreim,George W. Williams,Margaret Wu +17 more
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Vallerie V. McLaughlin,Stephen L. Archer,David B. Badesch,Robyn J. Barst,Harrison W. Farber,Jonathan R. Lindner,Michael A. Mathier,Michael D. McGoon,Myung H. Park,Robert S. Rosenson,Lewis J. Rubin,Victor F. Tapson,John Varga +12 more
TL;DR: In this paper, the authors discuss the role of the chairperson of FAHA and the chair's role in the FAHA Board of Trustee selection process and propose a new chairperson for FAHA.
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