CAMPHOR score: patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension

Abstract

BackgroundPulmonary endarterectomy (PEA) is the recommended treatment for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH). The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) score is an internationally-validated patient-reported outcome (PRO) measure for CTEPH. It assesses 3 domains: activity, quality of life (QoL) and symptoms. We assessed PROs in patients with CTEPH undergoing PEA.MethodsThis retrospective observational study of consecutive CTEPH patients undergoing PEA at the UK national PEA centre between 2006 and 2017 assessed change in CAMPHOR score from baseline (pre-PEA) until up to 5 years post-PEA. CAMPHOR scores were compared between (i) those with and without clinically significant residual PH and (ii) those undergoing PEA and propensity-matched CTEPH patients who were not operated on. The minimally clinically important difference (MCID) was calculated using an anchor-based method.ResultsOf 1324 CTEPH patients who underwent PEA, 1053 (80%) had a CAMPHOR score recorded pre-PEA, 934 (71%) within a year of PEA and 784 (60%) had both. There were significant improvements between pre- and post-PEA in all three CAMPHOR domains (median±interquartile range: activity, −5±7; QoL, −4±8; and symptoms, −7±8; p<0.0001, all). Improvements in CAMPHOR score were greater and more sustained in those without clinically significant residual PH. CTEPH patients undergoing PEA had better CAMPHOR scores than those not operated on. The MCID in CAMPHOR score was −3±5 for activity, −4±7 for QoL, and −6±7 for symptoms.ConclusionsPROs are markedly improved by PEA in patients with CTEPH, more so in those without clinically significant residual PH.

Health Related Quality of Life and Hospitalizations In Chronic Thromboembolic Pulmonary Hypertension Versus Idiopathic Pulmonary Arterial Hypertension: An Analysis from the Pulmonary Hypertension Association (PHAR)

“…(3, 23, 24) Studies exploring differences in HRQoL between patients with CTEPH and IPAH have mixed results with some finding no difference and others showing CTEPH patients having worse HRQoL. (13,25,26) The results from this study demonstrate that patients with CTEPH have worse HRQoL at referral to a Pulmonary Hypertension Care Center as compared to patients with IPAH, but these differences do not persist over time in a large prospective multicenter cohort. Among CTEPH patients, similar to what has been previously reported, (26) this study found that patients who undergo PTE have significant improvement in their HRQoL, and the improvement in scores may be more significant in patients without residual disease post-PTE.…”

“…(13,25,26) The results from this study demonstrate that patients with CTEPH have worse HRQoL at referral to a Pulmonary Hypertension Care Center as compared to patients with IPAH, but these differences do not persist over time in a large prospective multicenter cohort. Among CTEPH patients, similar to what has been previously reported, (26) this study found that patients who undergo PTE have significant improvement in their HRQoL, and the improvement in scores may be more significant in patients without residual disease post-PTE. (4,5) The worse HRQoL observed in CTEPH patients at referral was not explained by older age as compared to IPAH patients; however, older age may be associated with increased co-morbid conditions, which were not available for us to account for.…”

Symptoms, impacts, and suitability of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire in patients with chronic thromboembolic pulmonary hypertension (CTEPH): a qualitative interview study

“…To the authors' knowledge, the only published PRO instruments for PH that assess both symptoms and impacts and whose validation work included patients with CTEPH are the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) [8] and the emPHasis-10 [9]. The CAMPHOR assesses symptoms, functioning, and HRQoL, and has been used to measure the symptoms and impacts of CTEPH [7]. However, the instrument includes 65 items [8,10], which could make it challenging to administer in both real-world clinical practice and clinical trials.…”

“…Patient-reported outcome (PRO) instruments are useful in evaluating CTEPH symptoms and their impact on patients’ daily lives and general wellbeing [ 6 ]. However, the limited number of clinical trials on CTEPH have not generally assessed HRQoL as an outcome, so there is little data pertaining to these outcomes in patients with CTEPH [ 6 , 7 ]. Those studies that have assessed HRQoL in patients with CTEPH have used PRO instruments that are either generic (e.g.…”

“…Those studies that have assessed HRQoL in patients with CTEPH have used PRO instruments that are either generic (e.g. Short Form-36 Health Survey [SF-36], EQ-5D) or that were designed to evaluate PH, respiratory disease, or heart failure [ 6 , 7 ]. There is a lack of evidence for the sensitivity of these instruments to adequately capture CTEPH-specific outcomes.…”