Patient-Reported Outcomes Assessed by the CAMPHOR Questionnaire Predict Clinical Deterioration in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

McCabe, Colm; Bennett, Maxine; Doughty, Natalie; Ross, Robert; Sharples, Linda D.; Pepke‐Zaba, Joanna

doi:10.1378/chest.12-2443

Cited by 50 publications

(48 citation statements)

References 28 publications

(21 reference statements)

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“…Th e baseline diff erences noted in both domains of the SF-36 and in response to tadalafi l remain novel fi ndings that need further exploration, particularly given the recent associations noted between HRQoL and clinical deterioration and survival. 20,21 Other characteristics were associated with odds of achieving clinically relevant outcomes. Older age at enrollment and shorter baseline 6MWD were both associated with attainment of clinically relevant responses.…”

Section: Discussionmentioning

confidence: 99%

“…18 Th e MID, defi ned as the smallest change or diff erence in an outcome measure, perceived as benefi cial, that would justify a change in the patient's medical management, was determined to be around 33 m. 19 Clinically relevant changes in HRQoL are also important in PAH and may predict clinical deterioration and survival. 20,21 Identifying clinical characteristics that are associated with clinically relevant improvements in intermediate measures in response to specifi c PAH therapy off ers the opportunity to tailor treatment strategies and to defi ne distinct disease phenotypes. Th erefore, we sought to defi ne patient characteristics associated with patient-important, clinically relevant changes in 6MWT and HRQoL, using data from the large clinical trial of tadalafi l in PAH.…”

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confidence: 99%

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Sex Differences in Response to Tadalafil in Pulmonary Arterial Hypertension

Mathai

Hassoun

Puhan

et al. 2015

Chest

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BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive disease with high rates of morbidity and mortality. Current therapies improve symptoms, functional capacity, and, in select cases, survival. Little is known about patient factors that may predict the likelihood of patient-important, clinically relevant responses to therapy such as the 6-min walk distance (6MWD) and health-related quality of life (HRQoL).

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Sex Differences in Response to Tadalafil in Pulmonary Arterial Hypertension

Mathai

Hassoun

Puhan

et al. 2015

Chest

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“…In a recent large retrospective study, McCabe and colleagues demonstrate the utility of the CAMPHOR for the prediction of clinical events in a cohort of patients with either IPAH or CTEPH (24). Baseline CAMPHOR scores were strongly predictive of clinical deterioration, defined as either need for escalation of therapy, hospital admission, lung transplantation, or death, overall and in both subgroups.…”

Section: Original Researchmentioning

confidence: 99%

“…One recent observational study in a mixed population of patients with IPAH and chronic thromboembolic pulmonary hypertension (CTEPH) demonstrated a significant relationship between baseline HRQOL using a disease-specific measure (Cambridge Pulmonary Hypertension Outcome Review [CAMPHOR]) and clinical deterioration, defined as need for additional PAH therapy, hospitalization, or death (24). Similarly, using the Minnesota Living with Heart Failure Questionnaire, a heart failurespecific tool, Cenedese and colleagues found a significant association between baseline HRQOL and survival in mixed cohort of patients with IPAH and CTEPH (25).…”

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confidence: 99%

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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“…While its measurement properties were carefully examined during the development process, its ''real-world'' performance in the hands of external investigators remains to be established. A recent study suggests that the CAMPHOR is predictive of clinical deterioration at diagnosis but is not predictive over time [19]. The development of a new instrument that can be quickly administered in a clinic setting and yields a single score has appeal, and provides researchers with greater choice in terms of study measures.…”

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confidence: 99%

Measuring health in pulmonary hypertension: emphasising the right end-point?

Gomberg‐Maitland

Chen²

2014

European Respiratory Journal

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@ERSpublications emPHasis-10 addresses the unmet need for disease-specific quality of life instruments for pulmonary hypertension http://ow.ly/tdPDV Pulmonary hypertension (PH) is a condition deriving from multiple aetiologies with the recent focus on Group 1 pulmonary arterial hypertension (PAH) and Group 4 chronic thromboembolic pulmonary hypertension (CTEPH) largely due to the surge of understanding in pathophysiology and resultant treatments available [1]. Both are a disease of the pulmonary circulation that is characterised by a progressive rise in pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR), with limited dysfunction of the left ventricle [2]. Symptoms manifest as patients' lose the ability to augment their cardiac output in the face of a fixed vascular resistance [3]. In addition, anxiety and depression disorders are highly prevalent in PAH and CTEPH patients and correlate with the patients' sense of wellness [4]. However, despite therapeutic options and overall improved outcomes, life expectancy is shortened [5,6] and patients must face the myriad of issues that go along with a chronic debilitating fatal disease [7][8][9].

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Patient-Reported Outcomes Assessed by the CAMPHOR Questionnaire Predict Clinical Deterioration in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

Abstract: When emphasis is placed on the evaluation of patient perceptions, CAMPHOR may represent an alternative method of estimating the likelihood of CD.

Cited by 50 publications

References 28 publications

Sex Differences in Response to Tadalafil in Pulmonary Arterial Hypertension

Sex Differences in Response to Tadalafil in Pulmonary Arterial Hypertension

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

Measuring health in pulmonary hypertension: emphasising the right end-point?

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