TY - JOUR
T1 - Pulmonary arterial hypertension
T2 - Updates in epidemiology and evaluation of patients
AU - Levine, Deborah Jo
N1 - Funding Information:
cine, pulmonary and critical care, and director of pulmonary hypertension, University of Texas Health, San Antonio, TX. Funding source: This activity is supported by an educational grant from United Therapeutics Corporation. Author disclosure: Dr Levine has no relevant financial relationships with commercial interests to disclose. Authorship information: Substantial contributions to concept and design; drafting of the manuscript; and critical revision of the manuscript for important intellectual content. Address correspondence to: levinedj@uthscsa.edu Medical writing and editorial support: Brittany Hoffmann-Eubanks, PharmD, MBA
Publisher Copyright:
© 2021 Ascend Media. All rights reserved.
PY - 2021/3
Y1 - 2021/3
N2 - Group 1 pulmonary hypertension (or pulmonary arterial hypertension) is a rare, highly complex, and progressive disorder that is incurable and ultimately can lead to premature death. PAH causes significant physical, social, work, and emotional burdens among affected patients and their caregivers. Early diagnosis and initiation of treatment is required for best outcomes; however, the clinical presentation of PAH is nonspecific and frequently overlaps with several other conditions, often leading to a delay in diagnosis or misdiagnosis. In the past decades, increased understanding of the pathobiology of PAH has led to changes in its definition. Additionally, contemporary PAH registries have shown greater survival rates among patients with PAH and have allowed for the development of risk calculator tools that are now used to drive therapeutic goals. To date, multiple PAH-specific therapies have been developed, and all currently target one of 3 pathways that contribute to the endothelial dysfunction pathogenesis of PAH (prostacyclin, endothelin, and nitric oxide pathways). Because PAH is classified into 7 subgroups, it is essential that individuals are grouped appropriately for the efficacy of treatment and avoidance of harm. As health-related quality of life for PAH is multifactorial, it is important that patients are involved in the clinical decision-making process and have access to multidisciplinary care. The purpose of this review is to update healthcare professionals on the management of PAH with the most current information on epidemiology, pathophysiology, clinical presentation, and diagnostic considerations.
AB - Group 1 pulmonary hypertension (or pulmonary arterial hypertension) is a rare, highly complex, and progressive disorder that is incurable and ultimately can lead to premature death. PAH causes significant physical, social, work, and emotional burdens among affected patients and their caregivers. Early diagnosis and initiation of treatment is required for best outcomes; however, the clinical presentation of PAH is nonspecific and frequently overlaps with several other conditions, often leading to a delay in diagnosis or misdiagnosis. In the past decades, increased understanding of the pathobiology of PAH has led to changes in its definition. Additionally, contemporary PAH registries have shown greater survival rates among patients with PAH and have allowed for the development of risk calculator tools that are now used to drive therapeutic goals. To date, multiple PAH-specific therapies have been developed, and all currently target one of 3 pathways that contribute to the endothelial dysfunction pathogenesis of PAH (prostacyclin, endothelin, and nitric oxide pathways). Because PAH is classified into 7 subgroups, it is essential that individuals are grouped appropriately for the efficacy of treatment and avoidance of harm. As health-related quality of life for PAH is multifactorial, it is important that patients are involved in the clinical decision-making process and have access to multidisciplinary care. The purpose of this review is to update healthcare professionals on the management of PAH with the most current information on epidemiology, pathophysiology, clinical presentation, and diagnostic considerations.
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U2 - 10.37765/AJMC.2021.88609
DO - 10.37765/AJMC.2021.88609
M3 - Article
C2 - 33710842
AN - SCOPUS:85102914712
VL - 27
SP - S35-S41
JO - American Journal of Managed Care
JF - American Journal of Managed Care
SN - 1088-0224
IS - 3
ER -