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

Shafazand, Shirin; Goldstein, Mary K.; Doyle, Ramona L.; Hlatky, Mark A.; Gould, Michael K.

doi:10.1378/chest.126.5.1452

Cited by 140 publications

(140 citation statements)

References 30 publications

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“…The impairments observed in our population of PAH patients are as severe (and in many respects more so) than those reported in studies of patients with other severely debilitating and life-threatening conditions such as spinal cord injury, interstitial lung disease or cancer unresponsive to therapy ( Figure 7) [20][21][22][23][24][25][26]. Indeed, Shafazand et al found their patients with PAH unhappy enough with their condition as to be willing to accept a significant risk of death in exchange for a potential cure [17].…”

Section: Discussionmentioning

confidence: 51%

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

Taichman

Shin

Hudd

et al. 2005

Chest

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Section: Discussionmentioning

confidence: 51%

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

Taichman

Shin

Hudd

et al. 2005

Chest

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“…Given the cost and time associated with developing new instruments, it is not uncommon for investigators to modify existing measures for use in less prevalent conditions, as seen in cystic fibrosis, idiopathic pulmonary fibrosis, and sarcoidosis (20)(21)(22). In the case of PAH, cardiacand respiratory-specific instruments have frequently been used given their emphasis on the role of dyspnea and activity limitation in the disablement process (8)(9)(10)23). The validity of such instruments in PAH, however, depends in part on the extent to which those aspects of the disease that are shared in common are considered meaningful and important to patients with PAH.…”

Section: Instruments Used To Assess Hrqol In Pahmentioning

confidence: 99%

“…Shafazand and coworkers studied 53 patients using both generic and cardiac-specific measures (23). Patients reported significant impairment in all domains of the NHP, including energy, emotional reaction, pain, physical mobility, sleep, and social isolation in comparison to population norms.…”

Section: Studies Of Hrqol In Pahmentioning

confidence: 99%

Health-related Quality of Life and Patient-reported Outcomes in Pulmonary Arterial Hypertension

Chen¹,

Taichman²,

Doyle³

2008

Proceedings of the American Thoracic Society

100

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Advances in our understanding of the basic pathophysiology of pulmonary arterial hypertension (PAH) has led to an expanding number of therapeutic options. The ultimate goals of therapy are to lengthen survival while improving symptoms and quality of life. A wealth of research in other conditions has established health-related quality of life (HRQoL) to be an important clinical endpoint. Until recently, however, little was known about HRQoL in PAH, and how best to measure it. Over the past few years, several studies have begun contributing to this growing area of research. Instruments used to assess HRQoL have varied between studies. The extent to which these instruments are valid in PAH depend on their specific measurement properties. In this article, we provide an overview of the different types of patient-reported outcomes (PROs) used in PAH, focusing in particular on the measurement of HRQoL. In the process, we review the current literature on HRQoL in PAH, summarize the available data from randomized controlled trials, and discuss the implications of these findings on future research. Despite significant progress, the study of HRQoL in PAH remains a nascent field relative to other conditions. As the use of PROs continues to increase, additional work will be needed to begin standardizing the reporting and interpretation of such outcomes in future clinical trials.

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“…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%

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

Cited by 140 publications

References 30 publications

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

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

Health-related Quality of Life and Patient-reported Outcomes in Pulmonary Arterial Hypertension

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

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