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.

No abstract

Several patient-reported outcome measures have been developed to assess health status in pulmonary arterial hypertension (PAH). The required change in instrument scores needed, to be seen as meaningful to the individual, however remain unknown. We sought to identify minimal clinically important differences (MCIDs) in the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and to validate these against objective markers of functional capacity. MCIDs were established from a discovery cohort (n = 129) of consecutive incident cases of idiopathic PAH with CAMPHOR scores recorded at treatment-naïve baseline and 6 – 9 months following PAH therapy. An independent validation cohort (n = 87) was used to verify MCIDs. Concurrent measures of functional capacity relative to CAMPHOR scores were collected. MCIDs were derived using anchor- and distributional-based approaches. In the discovery cohort, mean (SD) age was 54.4 (16.4) years and 64% were female. Most patients (63%) were treated with sequential PAH therapy. Baseline CAMPHOR scores were: Symptoms, 12 (7); Activity, 12 (7) and QoL, 10 (7). PAH treatment resulted in significant improvements in CAMPHOR scores (p < 0.05). CAMPHOR MCIDs averaged across methods for health status improvement were: Symptoms, -4 points; Activity, -4 points and QoL -3 points. CAMPHOR Activity score change >= MCID with PAH treatment was associated with significantly greater improvement in 6-minute walk distance, in both discovery and validation cohorts. In conclusion, CAMPHOR scores are responsive to improvements in functional capacity with PAH treatment. MCIDs in PAH scales may provide useful insights into treatment response in future clinical trials.