TY - JOUR
T1 - Results of an Expert Consensus Survey on the Treatment of Pulmonary Arterial Hypertension With Oral Prostacyclin Pathway Agents
AU - McLaughlin, Vallerie V.
AU - Channick, Richard
AU - De Marco, Teresa
AU - Farber, Harrison W.
AU - Gaine, Sean
AU - Galié, Nazzareno
AU - Krasuski, Richard A.
AU - Preston, Ioana
AU - Souza, Rogerio
AU - Coghlan, J. Gerry
AU - Frantz, Robert P.
AU - Hemnes, Anna
AU - Kim, Nick H.
AU - Lang, Irene M.
AU - Langleben, David
AU - Li, Mengtao
AU - Sitbon, Olivier
AU - Tapson, Victor
AU - Frost, Adaani
N1 - Publisher Copyright:
© 2019 The Authors
PY - 2020/4
Y1 - 2020/4
N2 - 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.
AB - 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.
KW - oral prostacyclin
KW - oral treprostinil
KW - prostacyclin pathway agent
KW - pulmonary arterial hypertension
KW - selexipag
KW - Pulmonary Arterial Hypertension/etiology
KW - Humans
KW - Antihypertensive Agents/pharmacology
KW - Critical Pathways/standards
KW - Consensus
KW - Epoprostenol/metabolism
KW - Needs Assessment
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U2 - 10.1016/j.chest.2019.10.043
DO - 10.1016/j.chest.2019.10.043
M3 - Article
C2 - 31738929
AN - SCOPUS:85077721402
SN - 0012-3692
VL - 157
SP - 955
EP - 965
JO - CHEST
JF - CHEST
IS - 4
ER -