Estimating a Minimally Important Difference in Pulmonary Arterial Hypertension Following Treatment With Sildenafil

Gilbert, Clare; Brown, Martin C.; Cappelleri, Joseph C.; Carlsson, Martin; McKenna, Stephen P.

doi:10.1378/chest.07-0275

Cited by 78 publications

(62 citation statements)

References 23 publications

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“…For instance, Hoekstra and colleagues report that a 10-unit decrease in physical function subscale (the minimal important difference [MID] for this parameter in LHF) was associated with an 8% increase risk of death at 3 years in LHF (HR, 1.08; 95% confidence interval [CI], 1.02-1.14 in multivariable models) (48). In our cohort, a clinically relevant change in physical functioning subscale, reported as 13 units by Gilbert and colleagues in a cohort of patients with PAH, would be associated with a 24% increased risk of death (HR, 1.24; 95% CI, 1.03-1.49) (52).…”

Section: Original Researchmentioning

confidence: 60%

Health-related Quality of Life and Survival in Pulmonary Arterial Hypertension

et al. 2016

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Rationale: Pulmonary arterial hypertension is a progressive disease with high morbidity and mortality despite advances in medical therapy. The relationship between patient-related outcomes, such as healthrelated quality of life (HRQOL), and survival is not well described.Objective: To assess the relationship between HRQOL and outcomes in patients with pulmonary arterial hypertension.Methods: Consecutive patients with right heart catheterization-proven pulmonary arterial hypertension who completed the Medical Outcomes Survey Short Form-36 survey (SF-36) were included. Demographic, clinical, physiological, and hemodynamic data were collected at baseline. Survival was assessed from the time of diagnosis of pulmonary arterial hypertension. Cox proportional hazard models were constructed to assess the relationship between HRQOL and transplant-free survival.Measurements and Main Results: Eighty-seven patients with pulmonary arterial hypertension were enrolled and followed prospectively for a median of 3.8 years. At baseline, HRQOL was significantly worse than U.S. normal values for six of eight domains of the SF-36. Several domains demonstrated moderate correlation (r value > 0.40) with 6-minute-walk distance and World Health Organization functional class; there were no significant associations with hemodynamics. In univariable Cox proportional hazard models, six of eight domains and both summary scores were significantly associated with survival. In multivariable models, adjusted for age, disease type, and cardiac function, these relationships largely persisted. Conclusions:In this cohort of patients with pulmonary arterial hypertension, HRQOL, as assessed by the SF-36, was strongly associated with transplant-free survival. These relationships persisted when controlling for potential confounders such as disease type and disease severity. These findings suggest that HRQOL may be an important predictor of outcomes in pulmonary arterial hypertension and therefore a target for future therapeutic interventions.

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Section: Original Researchmentioning

confidence: 60%

Health-related Quality of Life and Survival in Pulmonary Arterial Hypertension

et al. 2016

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“…79 However, as previously noted, in SSc-PAH, 6MWD as a surrogate marker for RV function may be confounded by the high prevalence of myopathy, arthropathy, and ILD. [80][81][82][83] Pro-BNP also offers some value as a longitudinal management tool.…”

Section: Role Of Sponsorsmentioning

confidence: 91%

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

Pavec

Humbert

Mouthon

et al. 2010

Am J Respir Crit Care Med

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Systemic sclerosis (SSc) is a complex, multisystem disease characterized by fi brosis and excessive collagen deposition within the skin and internal organs, chronic infl ammation, autoimmune dysregulation, and microvascular endothelial dysfunction. There are two forms of SSc that are characterized by the extent of skin involvement. Limited cutaneous SSc predominantly involves the skin of the hands, arms, and face, and diffuse SSc involves large areas of skin and multiple organs. 1 Both forms are systemic diseases associated with signifi cant morbidity and mortality. Historically, mortality was largely caused by renal disease. With the advent of angiotensin-converting enzyme inhibitors to treat SSc renal crisis, SSc-associated pulmonary arterial hypertension (SSc-PAH) has emerged as a leading cause of morbidity and mortality, accounting for up to 30% of premature deaths. 2 Although SSc-PAH represents a signifi cant proportion of the pulmonary arterial hypertension (PAH) population (15%-30%), 3,4 much of what we know about PAH is derived from studies of idiopathic PAH (IPAH).Pulmonary arterial hypertension (PAH) is the leading cause of death in systemic sclerosis (SSc ) and affects up to 12% of all patients with SSc, with a 50% mortality rate within 3 years of PAH diagnosis. Compared with the idiopathic form of PAH (IPAH), patients with SSc-associated PAH (SSc-PAH) have a threefold increased risk of death and may receive a diagnosis late in the course of disease because of insidious onset and the high prevalence of cardiac, musculoskeletal, and pulmonary parenchymal comorbidities. Treatment with conventional forms of PAH therapy often yield poor results compared with IPAH cohorts; unfortunately, the exact reasons behind this remain poorly understood but likely include variations in the pathologic mechanisms, differences in cardiovascular response to increasing afterload, and inadequate strategies to detect and treat SSc-PAH early in its course. Current methods for screening and longitudinal evaluation of SSc-PAH, such as the 6-min walk test, transthoracic echocardiography, and MRI, each have notable advantages and disadvantages. We provide an up-to-date, focused review of SSc-PAH and how it differs from IPAH, including pathogenesis, appropriate screening for disease onset, and new approaches to treatment and longitudinal assessment of this disease. CHEST 2013; 144(4):1346-1356Abbreviations: 6MWD 5 6-min walk distance; CCB 5 calcium channel blocker; CTD 5 connective tissue disease; D lco 5 diffusing capacity of lung for carbon monoxide; EIPAH 5 exercise-induced pulmonary arterial hypertension; ERA 5 endothelin receptor antagonist; FC 5 functional class; ILD 5 interstitial lung disease; IPAH 5 idiopathic pulmonary arterial hypertension; LV 5 left ventricular; mPAP 5 mean pulmonary artery pressure; PAH 5 pulmonary arterial hypertension; PASP 5 pulmonary artery systolic pressure; PDE-5I 5 phosphodiesterase type 5 inhibitor; PFT 5 pulmonary function test; PH 5 pulmonary hypertension; pro-BNP 5 pro-brain natriuretic pe...

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“…The learning effect may be attenuated over subsequent repeats of the test [7]. WISE [192] PUHAN [193] PUHAN [194] POLKEY [195] GILBERT [197] MATHAI [198] HOLLAND [85] SWIGRIS [196] DU BOIS [62] However, there is a suggestion that over longer periods (.8 weeks) the learning effect may reset to a level similar to that recorded at first test exposure [208]. This observation requires further corroboration.…”

Section: Learning Effect On Iswtmentioning

confidence: 99%

An official systematic review of the European Respiratory Society/American Thoracic Society: measurement properties of field walking tests in chronic respiratory disease

Singh¹,

Puhan²,

et al. 2014

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This systematic review examined the measurement properties of the 6-min walk test (6MWT), incremental shuttle walk test (ISWT) and endurance shuttle walk test (ESWT) in adults with chronic respiratory disease.Studies that report the evaluation or use of the 6MWT, ISWT or ESWT were included. We searched electronic databases for studies published between January 2000 and September 2013.The 6-min walking distance (6MWD) is a reliable measure (intra-class correlation coefficients ranged from 0.82 to 0.99 in seven studies). There is a learning effect, with greater distance walked on the second test (pooled mean improvement of 26 m in 13 studies). Reliability was similar for ISWT and ESWT, with a learning effect also evident for ISWT (pooled mean improvement of 20 m in six studies). The 6MWD correlates more strongly with peak work capacity (r50.59-0.93) and physical activity (r50.40-0.85) than with respiratory function (r50.10-0.59). Methodological factors affecting 6MWD include track length, encouragement, supplemental oxygen and walking aids. Supplemental oxygen also affects ISWT and ESWT performance. Responsiveness was moderate to high for all tests, with greater responsiveness to interventions that included exercise training.The findings of this review demonstrate that the 6MWT, ISWT and ESWT are robust tests of functional exercise capacity in adults with chronic respiratory disease. OverviewThe aim of this systematic review was to examine the measurement properties for the 6-min walk test (6MWT), incremental shuttle walk test (ISWT) and endurance shuttle walk test (ESWT) in adults with chronic respiratory disease. A companion paper describes the standard operating procedures for the tests [1]. The key findings of this systematic review are as follows.1) The 6-min walking distance (6MWD) is a valid and reliable measure of exercise capacity for people with chronic lung disease. The 6MWD correlates more strongly with measures of peak work capacity and physical activity than with respiratory function or quality of life, which supports its conceptualisation as a test of functional exercise performance.2) The ISWT offers a different protocol to the 6MWT as it is incremental and externally paced. The ISWT is a valid and reliable measure of cardiopulmonary exercise capacity in chronic obstructive pulmonary disease (COPD), where there is a strong relationship between ISWT distance and peak oxygen uptake (V9O 2 peak) or work rate on a cardiopulmonary exercise test (CPET).3) A learning effect is observed for the 6MWT and the ISWT. The second test usually is the better compared with the first, but this is inconsistent.4) The ESWT is a test of endurance capacity. It is externally paced and is performed along the same course as the ISWT. Two tests do not appear to be necessary if the second test is conducted on the same day.5) Reference equations have been proposed for the 6MWD and the ISWT. Age, height and weight are included in most equations. The influence of race and ethnicity is unclear.6) The safety profile of ...

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Estimating a Minimally Important Difference in Pulmonary Arterial Hypertension Following Treatment With Sildenafil

Cited by 78 publications

References 23 publications

Health-related Quality of Life and Survival in Pulmonary Arterial Hypertension

Health-related Quality of Life and Survival in Pulmonary Arterial Hypertension

Systemic Sclerosis-associated Pulmonary Arterial Hypertension

An official systematic review of the European Respiratory Society/American Thoracic Society: measurement properties of field walking tests in chronic respiratory disease

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