James C Coons1, Kristen Pogue2, Andrew R Kolodziej3, Glenn A Hirsch4, Marjorie Patricia George5. 1. Department of Pharmacy and Therapeutics, University of Pittsburgh School of Pharmacy, UPMC Presbyterian Hospital, 727 Salk Hall, 3501 Terrace Street, Pittsburgh, PA, 15261, USA. coonsjc@upmc.edu. 2. University of Michigan, Ann Arbor, MI, USA. 3. Gill Heart Institute, University of Kentucky, Lexington, KY, USA. 4. Division of Cardiology, Department of Medicine, National Jewish Health, Denver, CO, USA. 5. Division of Pulmonary, Critical Care & Sleep Medicine, Department of Medicine, National Jewish Health, Denver, CO, USA.
Abstract
PURPOSE OF REVIEW: Pulmonary arterial hypertension (PAH) leads to progressive increases in pulmonary vascular resistance (PVR), right heart failure, and death if left untreated. This review will summarize and discuss recent updates in the classification and management of patients with PAH. RECENT FINDINGS: PAH requires careful hemodynamic assessment and is defined by a mean pulmonary artery pressure > 20 mmHg with normal left-sided filling pressures and a PVR ≥ 3 Wood units. Most patients with PAH require targeted pharmacotherapy based on multiparametric risk stratification. Significant improvements in clinical outcome have been realized through the approval of 14 unique pharmacotherapeutic options. The latest clinical recommendations provide the updated hemodynamic definition and clinical classification as well as evidence-based treatment recommendations. An important change is the focus on initial upfront combination therapy for most patients with PAH. Structured follow-up and escalation of treatment for those not achieving low-risk status is paramount.
PURPOSE OF REVIEW: Pulmonary arterial hypertension (PAH) leads to progressive increases in pulmonary vascular resistance (PVR), right heart failure, and death if left untreated. This review will summarize and discuss recent updates in the classification and management of patients with PAH. RECENT FINDINGS:PAH requires careful hemodynamic assessment and is defined by a mean pulmonary artery pressure > 20 mmHg with normal left-sided filling pressures and a PVR ≥ 3 Wood units. Most patients with PAH require targeted pharmacotherapy based on multiparametric risk stratification. Significant improvements in clinical outcome have been realized through the approval of 14 unique pharmacotherapeutic options. The latest clinical recommendations provide the updated hemodynamic definition and clinical classification as well as evidence-based treatment recommendations. An important change is the focus on initial upfront combination therapy for most patients with PAH. Structured follow-up and escalation of treatment for those not achieving low-risk status is paramount.
Entities:
Keywords:
Chronic thromboembolic pulmonary hypertension (CTEPH); Pulmonary arterial hypertension; Pulmonary hypertension; WHO group 1 pulmonary hypertension
Authors: Marius M Hoeper; Harm Jan Bogaard; Robin Condliffe; Robert Frantz; Dinesh Khanna; Marcin Kurzyna; David Langleben; Alessandra Manes; Toru Satoh; Fernando Torres; Martin R Wilkins; David B Badesch Journal: J Am Coll Cardiol Date: 2013-12-24 Impact factor: 24.094
Authors: Hanno H Leuchte; Michal El Nounou; Juergen Christian Tuerpe; Bertram Hartmann; Rainer A Baumgartner; Michael Vogeser; Olaf Muehling; Jürgen Behr Journal: Chest Date: 2007-02 Impact factor: 9.410
Authors: Gerald Simonneau; Robyn J Barst; Nazzareno Galie; Robert Naeije; Stuart Rich; Robert C Bourge; Anne Keogh; Ronald Oudiz; Adaani Frost; Shelmer D Blackburn; James W Crow; Lewis J Rubin Journal: Am J Respir Crit Care Med Date: 2002-03-15 Impact factor: 21.405
Authors: Xavier Jaïs; Andrea M D'Armini; Pavel Jansa; Adam Torbicki; Marion Delcroix; Hossein A Ghofrani; Marius M Hoeper; Irene M Lang; Eckhard Mayer; Joanna Pepke-Zaba; Loïc Perchenet; Adele Morganti; Gérald Simonneau; Lewis J Rubin Journal: J Am Coll Cardiol Date: 2008-12-16 Impact factor: 24.094
Authors: Victor F Tapson; Zhi-Cheng Jing; Kai-Feng Xu; Lei Pan; Jeremy Feldman; David G Kiely; Eugene Kotlyar; C Shane McSwain; Kevin Laliberte; Carl Arneson; Lewis J Rubin Journal: Chest Date: 2013-09 Impact factor: 9.410
Authors: Francisca Alexandra Gavilanes-Oleas; Jose Leonidas Alves; Caio Julio Cesar Fernandes; Luis Felipe Lopes Prada; William Salibe Filho; Mario Terra Filho; Luciana Morinaga; Susana Hoette; Carlos Jardim; Rogerio Souza Journal: Clinics (Sao Paulo) Date: 2018-05-17 Impact factor: 2.365
Authors: Nazzareno Galiè; Richard N Channick; Robert P Frantz; Ekkehard Grünig; Zhi Cheng Jing; Olga Moiseeva; Ioana R Preston; Tomas Pulido; Zeenat Safdar; Yuichi Tamura; Vallerie V McLaughlin Journal: Eur Respir J Date: 2019-01-24 Impact factor: 16.671
Authors: Nick H Kim; Marion Delcroix; Xavier Jais; Michael M Madani; Hiromi Matsubara; Eckhard Mayer; Takeshi Ogo; Victor F Tapson; Hossein-Ardeschir Ghofrani; David P Jenkins Journal: Eur Respir J Date: 2019-01-24 Impact factor: 16.671
Authors: James C Coons; Karryn Crisamore; Solomon Adams; Ashley Modany; Marc A Simon; Wenchen Zhao; Imam H Shaik; Raman Venkataramanan; Philip E Empey Journal: Ther Adv Respir Dis Date: 2021 Jan-Dec Impact factor: 4.031