Abstract
Background Pulmonary 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 three domains: activity, quality of life (QoL) and symptoms. We assessed PROs in patients with CTEPH undergoing PEA.
Methods This 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 1) those with and without clinically significant residual pulmonary hypertension and 2) 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.
Results Out of 1324 CTEPH patients who underwent PEA, 1053 (80%) had a CAMPHOR score recorded pre-PEA, 934 (71%) had a score recorded 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, symptoms −7±8; all p<0.0001). Improvements in CAMPHOR score were greater and more sustained in those without clinically significant residual pulmonary hypertension. 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.
Conclusions PROs are markedly improved by PEA in patients with CTEPH, more so in those without clinically significant residual pulmonary hypertension.
Abstract
Patients with CTEPH report significant improvement in patient-reported CAMPHOR scores after pulmonary endarterectomy compared with patients not operated on, but those with clinically significant residual pulmonary hypertension have less benefit https://bit.ly/2yU8V1v
Footnotes
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Conflict of interest: M. Newnham has nothing to disclose.
Conflict of interest: K. Bunclark has nothing to disclose.
Conflict of interest: N. Abraham has nothing to disclose.
Conflict of interest: S. Ali has nothing to disclose.
Conflict of interest: L. Amaral-Almeida has nothing to disclose.
Conflict of interest: J.E. Cannon reports grants from Actelion, during the conduct of the study; meeting travel support from Actelion and GSK, outside the submitted work.
Conflict of interest: N. Doughty has nothing to disclose.
Conflict of interest: C. Ng has nothing to disclose.
Conflict of interest: A. Ponnaberanam has nothing to disclose.
Conflict of interest: K. Sheares reports educational support from Actelion, Bayer and GSK, personal fees for consultancy from Actelion, outside the submitted work.
Conflict of interest: N. Speed has nothing to disclose.
Conflict of interest: D. Taboada reports other (speaker honoraria and education/travel grants) from Actelion, Bayer, GlaxoSmithKline, Lilly, MDS and Pfizer, outside the submitted work.
Conflict of interest: M. Toshner reports personal fees from Actelion/J&J and GSK, grants and personal fees from Bayer, grants from Merck, outside the submitted work.
Conflict of interest: S. Tsui has nothing to disclose.
Conflict of interest: D.P. Jenkins reports grants and personal fees for lectures from Bayer, personal fees for lectures and adjudication committee work from Actelion, outside the submitted work.
Conflict of interest: J. Pepke-Zaba has participated in advisory board activities for Actelion, GSK and MSD, given paid lectures for Actelion and MSD, and via institution has received research, education grants from Actelion and MSD.
Support statement: M. Toshner is supported by the National Institute for Health Research Cardiorespiratory Biomedical Research Centre.
- Received October 26, 2019.
- Accepted May 7, 2020.
- Copyright ©ERS 2020