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Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”

Women with pulmonary arterial hypertension (PAH) experience multiple symptoms, including dyspnea, fatigue, and sleep disturbance, that impair their health‐related quality of life (HRQOL). However, we know little about phenotypic subgroups of patients with PAH with similar, concurrent, multiple sympt...

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Autores principales: Matura, Lea Ann, Fargo, Jamison D., Boyle, Kathleen, Fritz, Jason S., Smith, Kerri A., Mazurek, Jeremy A., Pinder, Diane, Archer‐Chicko, Christine L., Palevsky, Harold I., Pack, Allan I., Sommers, Marilyn S., Kawut, Steven M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9511227/
https://www.ncbi.nlm.nih.gov/pubmed/36186717
http://dx.doi.org/10.1002/pul2.12135
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author Matura, Lea Ann
Fargo, Jamison D.
Boyle, Kathleen
Fritz, Jason S.
Smith, Kerri A.
Mazurek, Jeremy A.
Pinder, Diane
Archer‐Chicko, Christine L.
Palevsky, Harold I.
Pack, Allan I.
Sommers, Marilyn S.
Kawut, Steven M.
author_facet Matura, Lea Ann
Fargo, Jamison D.
Boyle, Kathleen
Fritz, Jason S.
Smith, Kerri A.
Mazurek, Jeremy A.
Pinder, Diane
Archer‐Chicko, Christine L.
Palevsky, Harold I.
Pack, Allan I.
Sommers, Marilyn S.
Kawut, Steven M.
author_sort Matura, Lea Ann
collection PubMed
description Women with pulmonary arterial hypertension (PAH) experience multiple symptoms, including dyspnea, fatigue, and sleep disturbance, that impair their health‐related quality of life (HRQOL). However, we know little about phenotypic subgroups of patients with PAH with similar, concurrent, multiple symptoms. The objectives of this study were to define the “symptome” by symptom cluster phenotypes and compare characteristics such as biomarkers, cardiac structure and function (echocardiography), functional capacity (6‐min walk distance), and HRQOL between the groups. This cross‐sectional study included 60 women with PAH. Subjects completed an assessment battery: Pulmonary Arterial Hypertension Symptom Scale, Pittsburgh Sleep Quality Index, Multidimensional Dyspnea Profile, Patient‐Reported Outcomes Measurement Information System (PROMIS®) Physical Function, PROMIS® Sleep‐Related Impairment, and the emPHasis‐10. Subjects also underwent transthoracic echocardiography, phlebotomy, 6‐min walk distance, and actigraphy. The three symptoms of dyspnea, fatigue, and sleep disturbance were used to define the symptom clusters. Other PAH symptoms, plasma and serum biomarkers, cardiac structure and function (echocardiography), exercise capacity (6‐min walk distance), sleep (actigraphy), and HRQOL were compared across phenotypes. The mean age was 50 ± 18 years, 51% were non‐Hispanic white, 32% were non‐Hispanic Black and 40% had idiopathic PAH. Cluster analysis identified Mild (n = 28, 47%), Moderate (n = 20, 33%), and Severe Symptom Cluster Phenotypes (n = 12, 20%). There were no differences for age, race, or PAH etiology between the phenotypes. WHO functional class (p < 0.001), norepinephrine levels (p = 0.029), right atrial pressure (p = 0.001), physical function (p < 0.001), sleep onset latency (p = 0.040), and HRQOL (p < 0.001) all differed significantly across phenotypes. We identified three distinctive symptom cluster phenotypes (Mild, Moderate, and Severe) for women with PAH that also differed by PAH‐related symptoms, physical function, right atrial pressure, norepinephrine levels, and HRQOL. These phenotypes could suggest targeted interventions to improve symptoms and HRQOL in those most severely affected.
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spelling pubmed-95112272022-09-30 Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome” Matura, Lea Ann Fargo, Jamison D. Boyle, Kathleen Fritz, Jason S. Smith, Kerri A. Mazurek, Jeremy A. Pinder, Diane Archer‐Chicko, Christine L. Palevsky, Harold I. Pack, Allan I. Sommers, Marilyn S. Kawut, Steven M. Pulm Circ Research Articles Women with pulmonary arterial hypertension (PAH) experience multiple symptoms, including dyspnea, fatigue, and sleep disturbance, that impair their health‐related quality of life (HRQOL). However, we know little about phenotypic subgroups of patients with PAH with similar, concurrent, multiple symptoms. The objectives of this study were to define the “symptome” by symptom cluster phenotypes and compare characteristics such as biomarkers, cardiac structure and function (echocardiography), functional capacity (6‐min walk distance), and HRQOL between the groups. This cross‐sectional study included 60 women with PAH. Subjects completed an assessment battery: Pulmonary Arterial Hypertension Symptom Scale, Pittsburgh Sleep Quality Index, Multidimensional Dyspnea Profile, Patient‐Reported Outcomes Measurement Information System (PROMIS®) Physical Function, PROMIS® Sleep‐Related Impairment, and the emPHasis‐10. Subjects also underwent transthoracic echocardiography, phlebotomy, 6‐min walk distance, and actigraphy. The three symptoms of dyspnea, fatigue, and sleep disturbance were used to define the symptom clusters. Other PAH symptoms, plasma and serum biomarkers, cardiac structure and function (echocardiography), exercise capacity (6‐min walk distance), sleep (actigraphy), and HRQOL were compared across phenotypes. The mean age was 50 ± 18 years, 51% were non‐Hispanic white, 32% were non‐Hispanic Black and 40% had idiopathic PAH. Cluster analysis identified Mild (n = 28, 47%), Moderate (n = 20, 33%), and Severe Symptom Cluster Phenotypes (n = 12, 20%). There were no differences for age, race, or PAH etiology between the phenotypes. WHO functional class (p < 0.001), norepinephrine levels (p = 0.029), right atrial pressure (p = 0.001), physical function (p < 0.001), sleep onset latency (p = 0.040), and HRQOL (p < 0.001) all differed significantly across phenotypes. We identified three distinctive symptom cluster phenotypes (Mild, Moderate, and Severe) for women with PAH that also differed by PAH‐related symptoms, physical function, right atrial pressure, norepinephrine levels, and HRQOL. These phenotypes could suggest targeted interventions to improve symptoms and HRQOL in those most severely affected. John Wiley and Sons Inc. 2022-07-01 /pmc/articles/PMC9511227/ /pubmed/36186717 http://dx.doi.org/10.1002/pul2.12135 Text en © 2022 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Research Articles
Matura, Lea Ann
Fargo, Jamison D.
Boyle, Kathleen
Fritz, Jason S.
Smith, Kerri A.
Mazurek, Jeremy A.
Pinder, Diane
Archer‐Chicko, Christine L.
Palevsky, Harold I.
Pack, Allan I.
Sommers, Marilyn S.
Kawut, Steven M.
Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”
title Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”
title_full Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”
title_fullStr Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”
title_full_unstemmed Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”
title_short Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”
title_sort symptom phenotypes in pulmonary arterial hypertension: the pah “symptome”
topic Research Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9511227/
https://www.ncbi.nlm.nih.gov/pubmed/36186717
http://dx.doi.org/10.1002/pul2.12135
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