Vallerie V McLaughlin1, Richard Channick2, Teresa De Marco3, Harrison W Farber4, Sean Gaine5, Nazzareno Galié6, Richard A Krasuski7, Ioana Preston4, Rogerio Souza8, J Gerry Coghlan9, Robert P Frantz10, Anna Hemnes11, Nick H Kim12, Irene M Lang13, David Langleben14, Mengtao Li15, Olivier Sitbon16, Victor Tapson17, Adaani Frost18. 1. Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI. Electronic address: vmclaugh@umich.edu. 2. Department of Medicine, University of California Los Angeles Medical Center, Los Angeles, CA. 3. Department of Cardiology, University of California, San Francisco, CA. 4. Division of Pulmonary, Critical Care and Sleep Medicine, Tufts Medical Center, Boston, MA. 5. The Mater Hospital, Dublin, Ireland. 6. Department of Experimental, Diagnostic and Specialty Medicine-DIMES, University of Bologna, Bologna, Italy. 7. Duke University Hospital, Durham, NC. 8. Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil. 9. Department of Cardiology, Royal Free Hospital, London, England. 10. Department of Cardiovascular Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN. 11. Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, TN. 12. Department of Cardiothoracic Surgery, University of California San Diego Medical Center, San Diego, CA. 13. Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna, Allgemeines Krankenhaus, Vienna, Austria. 14. Center for Pulmonary Vascular Disease, Cardiology Division, Jewish General Hospital and McGill University, Montreal, QC, Canada. 15. Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China. 16. Université Paris-Saclay, APHP, Hôpital Bicêtre, Service de Pneumologie, Le Kremlin-Bicêtre, France. 17. Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA. 18. Department of Medicine, Institute of Academic Medicine, Houston Methodist Hospital, Houston, TX.
Abstract
BACKGROUND: Treatment of pulmonary arterial hypertension (PAH) has evolved substantially over the past two decades and varies according to etiology, functional class (FC), hemodynamic parameters, and other clinical factors. Current guidelines do not provide definitive recommendations regarding the use of oral prostacyclin pathway agents (PPAs) in PAH. To provide guidance on the use of these agents, an expert panel was convened to develop consensus statements for the initiation of oral PPAs in adults with PAH. METHODS: A systematic literature search was conducted using MEDLINE. The established RAND/University of California Los Angeles appropriateness method, which incorporates the Delphi method and the nominal group technique, was used to create consensus statements. Idiopathic, heritable, repaired congenital heart defect, and drug- or toxin-induced PAH (IPAH+) was considered as one etiologic grouping. The process was focused on the use of oral treprostinil or selexipag in patients with IPAH+ or connective tissue disease-associated PAH and FC II or III symptoms receiving background dual endothelin receptor antagonist/phosphodiesterase type 5 inhibitor therapy. RESULTS: The panel developed 14 consensus statements regarding the appropriate use of oral PPAs in the target population. The panel identified 13 clinical scenarios in which selexipag may be considered as a treatment option. CONCLUSIONS: The paucity of clinical evidence overall, and particularly from randomized trials in this setting, creates a gap in knowledge. These consensus statements are intended to aid physicians in navigating treatment options and using oral PPAs in the most appropriate manner in patients with PAH.
BACKGROUND: Treatment of pulmonary arterial hypertension (PAH) has evolved substantially over the past two decades and varies according to etiology, functional class (FC), hemodynamic parameters, and other clinical factors. Current guidelines do not provide definitive recommendations regarding the use of oral prostacyclin pathway agents (PPAs) in PAH. To provide guidance on the use of these agents, an expert panel was convened to develop consensus statements for the initiation of oral PPAs in adults with PAH. METHODS: A systematic literature search was conducted using MEDLINE. The established RAND/University of California Los Angeles appropriateness method, which incorporates the Delphi method and the nominal group technique, was used to create consensus statements. Idiopathic, heritable, repaired congenital heart defect, and drug- or toxin-induced PAH (IPAH+) was considered as one etiologic grouping. The process was focused on the use of oral treprostinil or selexipag in patients with IPAH+ or connective tissue disease-associated PAH and FC II or III symptoms receiving background dual endothelin receptor antagonist/phosphodiesterase type 5 inhibitor therapy. RESULTS: The panel developed 14 consensus statements regarding the appropriate use of oral PPAs in the target population. The panel identified 13 clinical scenarios in which selexipag may be considered as a treatment option. CONCLUSIONS: The paucity of clinical evidence overall, and particularly from randomized trials in this setting, creates a gap in knowledge. These consensus statements are intended to aid physicians in navigating treatment options and using oral PPAs in the most appropriate manner in patients with PAH.
Authors: Benjamin Daines; Sanjana Rao; Omid Hosseini; Sofia Prieto; John Abdelmalek; Mohamed Elmassry; Pooja Sethi; Victor Test; Kenneth Nugent Journal: J Community Hosp Intern Med Perspect Date: 2021-11-15
Authors: Sean X Gu; Tarun Tyagi; Kanika Jain; Vivian W Gu; Seung Hee Lee; Jonathan M Hwa; Jennifer M Kwan; Diane S Krause; Alfred I Lee; Stephanie Halene; Kathleen A Martin; Hyung J Chun; John Hwa Journal: Nat Rev Cardiol Date: 2020-11-19 Impact factor: 32.419