Health-Related Quality of Life and Psychological States in Patients With Pulmonary Arterial Hypertension

Matura, Lea Ann; McDonough, Annette; Carroll, Diane L.

doi:10.1097/jcn.0b013e318275330d

Cited by 46 publications

(48 citation statements)

References 37 publications

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“…Not only does SSc-PAH affect survival, it also has a significant impact on patients’ functional capacity and HRQoL [15, 16]. Functional limitations, as captured by the SHAQ, were maintained over time with PAH therapy in our cohort, without a significant improvement in any specific domain, which is consistent with the literature [33, 34].…”

Section: Discussionsupporting

confidence: 87%

“…Survival in incident SSc-PAH may be below this as these figures are derived from incident and prevalent SSc-PAH cohort data, introducing a survival bias. Not only does SSc-PAH affect patient survival, it also has a significant impact on patients’ functional capacity and health-related quality of life (HRQoL) [15, 16]. We sought to determine survival and HRQoL related to incident SSc-PAH in a large cohort of Australian SSc patients, and to identify predictors of mortality.…”

Section: Introductionmentioning

confidence: 99%

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Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

et al. 2017

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BackgroundPulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH.MethodsWe studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality. HRQoL was measured using the Short form 36 (SF-36) instrument.ResultsAmong 132 SSc-PAH patients (112 female (85%); mean age 62 ± 11 years), 60 (45.5%) died, with a median (±IQR) survival time from PAH diagnosis of 4.0 (2.2–6.2) years. Median (±IQR) follow up from study enrolment was 3.8 (1.6–5.8) years. The SMR for patients with SSc-PAH was 5.8 (95% CI 4.3–7.8), with YLL of 15.2 years (95% CI 12.3–18.1). Combination PAH therapy had a survival advantage (p < 0.001) compared with monotherapy, as did anticoagulation compared with no anticoagulation (p < 0.003). Furthermore, combination PAH therapy together with anticoagulation had a survival benefit compared with monotherapy with or without anticoagulation and combination therapy without anticoagulation (hazard ratio 0.28, 95% CI 0.1–0.7). Older age at PAH diagnosis (p = 0.03), mild co-existent interstitial lung disease (ILD) (p = 0.01), worse WHO functional class (p = 0.03) and higher mean pulmonary arterial pressure at PAH diagnosis (p = 0.001), and digital ulcers (p = 0.01) were independent predictors of mortality.ConclusionsDespite the significant benefits conferred by advanced PAH therapies suggested in this study, the median survival in SSc PAH remains short at only 4 years.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-017-1341-x) contains supplementary material, which is available to authorized users.

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

et al. 2017

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“…An important point to note is that even symptoms that were not reported as severe still interfered with the patient’s life. In PAH, syncope is not necessarily a commonly reported symptom or a severe symptom (37). However, syncope is a poor prognostic sign in patients with PAH and needs to be assessed.…”

Section: Discussionmentioning

confidence: 99%

“…Additionally, as a result of vasoconstriction and plexiform lesions in the pulmonary vasculature, hypoxemia can result (3). Those patients with PAH with more physical limitations tend to be more likely to have advanced disease and be classified as disabled (37). Dizziness can limit the ability of patients to stand and move safely, putting them at risk for falls.…”

Section: Discussionmentioning

confidence: 99%

Symptom Interference Severity and Health-Related Quality of Life in Pulmonary Arterial Hypertension

Matura

McDonough

Carroll

2016

Journal of Pain and Symptom Management

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Context While assessing symptom severity is an important component of evaluating symptoms, understanding those symptoms that interfere with patients’ lives is also key. Pulmonary arterial hypertension (PAH) is a chronic disease resulting in right heart failure and increased mortality. Patients with PAH experience multiple symptoms but we do not know which symptoms and to what extent their symptoms interfere with daily life. Objectives To: 1) describe the prevalence of those symptoms that interfere with life; 2) describe the severity of symptom interference; and 3) determine those sociodemographic and clinical characteristics, and interfering symptoms associated with health-related quality of life (HRQOL) in patients with PAH. Methods A convenience sample of 191 patients with PAH completed a sociodemographic form; the Pulmonary Arterial Hypertension Symptom Interference Scale (PAHSIS) and the Medical Outcomes Survey Short Form-36 (SF-36) to measure HRQOL. Hierarchical multiple linear regression was used to analyze demographic and medical characteristics along with symptom interference from the PAHSIS as predictors of HRQOL from the composite mental and physical health summary scores of the SF-36. Results The most interfering symptoms reported were fatigue, shortness of breath with exertion and difficulty sleeping. Age, gender, functional class, oxygen use, fatigue, dizziness and Raynaud’s phenomenon were associated with the HRQOL physical health summary scores. The symptoms fatigue and SOB while lying down were associated with the HRQOL mental health summary scores. Conclusion Patients with PAH are experiencing multiple symptoms that are interfering with their HRQOL and ability to function.

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“…In Phase I, the PAHSIS was developed by the investigators from the literature by the research team which included clinicians with expertise caring for patients with PAH. The tool coincides with the same symptoms contained on the Pulmonary Arterial Hypertension Symptom Scale (PAHSS; Matura, McDonough, Hanlon, & Carroll, 2015). Symptoms include those commonly found in patients with cardiopulmonary disorders including various types of shortness of breath (SOB; e.g., exertional, lying down).…”

Section: Procedures For Instrument Developmentmentioning

confidence: 99%

Developmental and Initial Validation of the Pulmonary Arterial Hypertension Symptom Interference Scale

Matura

McDonough²,

Hanlon

et al. 2016

J Nurs Meas

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Health-Related Quality of Life and Psychological States in Patients With Pulmonary Arterial Hypertension

Abstract: Patients with PAH are experiencing diminished physical health and HRQOL. Future studies are needed to design and test interventions to improve HRQOL.

Cited by 46 publications

References 37 publications

Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

Symptom Interference Severity and Health-Related Quality of Life in Pulmonary Arterial Hypertension

Developmental and Initial Validation of the Pulmonary Arterial Hypertension Symptom Interference Scale

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