Comparison and validation of three measures of quality of life in patients with pulmonary hypertension

Chua, R.; Keogh, Anne; Byth, Karen; O'Loughlin, Aiden

doi:10.1111/j.1445-5994.2006.01169.x

Cited by 63 publications

(72 citation statements)

References 23 publications

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“…On the other hand, hemodynamics had no association with either the physical or mental component scores (discriminant validity). Investigators have shown similar correlations between the physical subscore of the modified MLHF questionnaire with functional class, 6MWD, RA pressure and CI [87,107]. The MLHF score was also associated with event-free survival [107].…”

Section: Quality Of Life (Qol)mentioning

confidence: 72%

“…A recent report substantiated the impact of changes in 6MWD on health status and QOL [87]. However, 6MWD may also be linked to survival in PAH, making it a surrogate end point as well.…”

Section: Exercise Testing Six Minute Walk Test (6mwt)mentioning

confidence: 99%

“…The MLHF score was also associated with event-free survival [107]. Chua et al have shown that improvements in 6MWD over time translate to improvements in QOL measured by the SF-36 [87].…”

Section: Quality Of Life (Qol)mentioning

confidence: 99%

“…Recent studies have initiated this validation process in PAH [87,104,106]. Taichman et al examined the SF-36 in reference to other components of disease activity in PAH [104].…”

Section: Quality Of Life (Qol)mentioning

confidence: 99%

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Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

Snow¹,

Kawut²

2007

Clinics in Chest Medicine

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Pulmonary arterial hypertension (PAH) is a rare disease which is characterized by increased pulmonary vascular resistance and right heart failure. Recent discoveries in disease pathophysiology have been translated into effective therapies tested in clinical trials. The studies which have led to the regulatory board approval of therapies for PAH have focused on surrogate and intermediate end points, thought to reflect quantity and quality of life, respectively. However, validation of many of these surrogates is incomplete. It is also unknown which indicators of function or long-term survival should be used to formulate decisions regarding addition, discontinuation, or combination of therapies. Identification of suitable end points would therefore not only help investigators design appropriate clinical trials, but also assist clinicians in caring for patients with PAH. Hemodynamic, cardiac imaging, plasma biomarkers, and exercise testing hold some promise as potential surrogate end points for PAH. Functional status and quality of life assessments may also have important roles. Future studies should validate the most promising surrogate markers, so that patients, clinicians, subjects, and investigators may benefit from the advantages they confer on clinical care and on clinical trials.The past several years have yielded an explosion of randomized clinical trials (RCTs) of several new medical therapies for pulmonary arterial hypertension (PAH). The hopes for these and all treatments for potentially fatal diseases are 1) to improve how a patient feels or functions and/ or 2) to prolong survival. While simply stated, the assessment of progress towards these goals may be quite complex [1][2][3][4][5][6].New PAH therapies have gained regulatory board approval based on studies with intermediate and surrogate end points as primary outcomes, believed to reflect the impact of these drugs on quality of life (QOL) and survival, respectively. While hemodynamic, plasma, cardiac imaging, exercise, functional, and QOL parameters are promising candidates, the reliability and validity of these measures are not clearly established. In addition, there are no RCTs addressing the use of such end points in guiding management decisions in PAH, such as changing, intensifying, escalating, or combining therapies. Despite this lack of evidence, clinicians who

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Section: Quality Of Life (Qol)mentioning

confidence: 72%

“…A recent report substantiated the impact of changes in 6MWD on health status and QOL [87]. However, 6MWD may also be linked to survival in PAH, making it a surrogate end point as well.…”

Section: Exercise Testing Six Minute Walk Test (6mwt)mentioning

confidence: 99%

“…The MLHF score was also associated with event-free survival [107]. Chua et al have shown that improvements in 6MWD over time translate to improvements in QOL measured by the SF-36 [87].…”

Section: Quality Of Life (Qol)mentioning

confidence: 99%

“…Recent studies have initiated this validation process in PAH [87,104,106]. Taichman et al examined the SF-36 in reference to other components of disease activity in PAH [104].…”

Section: Quality Of Life (Qol)mentioning

confidence: 99%

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Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

Snow¹,

Kawut²

2007

Clinics in Chest Medicine

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“…It also correlates with New York Heart Association Functional Class [127] and quality of life [129]. Similarly change in six minute walk distance (Δ6MWD) following PAH treatment correlated with changes in cardiac index, PVR [130] and quality of life [129].…”

Section: Field Exercise Testsmentioning

confidence: 97%

The Role of Exercise Testing in the Modern Management of Pulmonary Arterial Hypertension

Johnson

Thomson

2014

Diseases

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A culture of exercise testing is firmly embedded in the management of pulmonary arterial hypertension (PAH) but its clinical relevance and utility have recently been under some debate. The six minute walk test (6MWT) has been used as a primary outcome measure to enable the licensing of many of the medications used for this condition. Recent reviews have questioned the validity of this test as a surrogate of clinical outcomes. At the same time, other questions are emerging where exercise testing may be the solution. With the rise in understanding of genetic markers of idiopathic PAH (IPAH), the screening of an otherwise healthy population for incipient pulmonary hypertension (PH) will be required. The proliferation in treatment choices and identification of populations with PH where PAH treatment is not indicated, such as left heart and lung disease, requires more definitive differentiation from patients with PAH. There is a continuing question about the existence and clinical relevance of exercise induced PAH as a cause of unexplained dyspnoea and fatigue and as a latent phase of resting PH. This review presents a summary and critical analysis of the current role of exercise testing in PAH and speculates on future trends.

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Parenteral Prostacyclin Use in Pulmonary Arterial Hypertension

Huston

Hemnes

2020

Pulmonary Hypertension

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Comparison and validation of three measures of quality of life in patients with pulmonary hypertension

Abstract: The MLwHF questionnaire and SF-36 are useful tools for the assessment of QoL in pulmonary hypertension and may be useful in the ongoing evaluation of QoL in the treatment and study of pulmonary hypertension.

Cited by 63 publications

References 23 publications

Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

The Role of Exercise Testing in the Modern Management of Pulmonary Arterial Hypertension

Parenteral Prostacyclin Use in Pulmonary Arterial Hypertension

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