Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”
Abstract 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, multi...
| Main Authors: | , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2022-07-01
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| Series: | Pulmonary Circulation |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/pul2.12135 |
| _version_ | 1811205272100667392 |
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| author | Lea Ann Matura Jamison D. Fargo Kathleen Boyle Jason S. Fritz Kerri A. Smith Jeremy A. Mazurek Diane Pinder Christine L. Archer‐Chicko Harold I. Palevsky Allan I. Pack Marilyn S. Sommers Steven M. Kawut |
| author_facet | Lea Ann Matura Jamison D. Fargo Kathleen Boyle Jason S. Fritz Kerri A. Smith Jeremy A. Mazurek Diane Pinder Christine L. Archer‐Chicko Harold I. Palevsky Allan I. Pack Marilyn S. Sommers Steven M. Kawut |
| author_sort | Lea Ann Matura |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-04-12T03:27:57Z |
| format | Article |
| id | doaj.art-14ebbf7c34364151a0b3af668bc8b757 |
| institution | Directory Open Access Journal |
| issn | 2045-8940 |
| language | English |
| last_indexed | 2024-04-12T03:27:57Z |
| publishDate | 2022-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj.art-14ebbf7c34364151a0b3af668bc8b7572022-12-22T03:49:37ZengWileyPulmonary Circulation2045-89402022-07-01123n/an/a10.1002/pul2.12135Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome”Lea Ann Matura0Jamison D. Fargo1Kathleen Boyle2Jason S. Fritz3Kerri A. Smith4Jeremy A. Mazurek5Diane Pinder6Christine L. Archer‐Chicko7Harold I. Palevsky8Allan I. Pack9Marilyn S. Sommers10Steven M. Kawut11School of Nursing University of Pennsylvania Philadelphia Pennsylvania USADepartment of Psychology, Emma Eccles Jones College of Education and Human Services Utah State University Logan Utah USADepartment of Nursing Thomas Jefferson University Philadelphia Pennsylvania USAPerelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USAPerelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USAPerelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USAInstitutional Review Board University of Pennsylvania Philadelphia Pennsylvania USAPenn Medicine University of Pennsylvania Health System Philadelphia Pennsylvania USAPerelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USAPerelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USASchool of Nursing University of Pennsylvania Philadelphia Pennsylvania USAPerelman School of Medicine University of Pennsylvania Philadelphia Pennsylvania USAAbstract 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.https://doi.org/10.1002/pul2.12135cluster analysissymptom managementsymptoms |
| spellingShingle | Lea Ann Matura Jamison D. Fargo Kathleen Boyle Jason S. Fritz Kerri A. Smith Jeremy A. Mazurek Diane Pinder Christine L. Archer‐Chicko Harold I. Palevsky Allan I. Pack Marilyn S. Sommers Steven M. Kawut Symptom phenotypes in pulmonary arterial hypertension: The PAH “symptome” Pulmonary Circulation cluster analysis symptom management symptoms |
| 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 | cluster analysis symptom management symptoms |
| url | https://doi.org/10.1002/pul2.12135 |
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