TY - JOUR T1 - CAMPHOR score: patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension (CTEPH) JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.02096-2019 SP - 1902096 AU - Michael Newnham AU - Katherine Bunclark AU - Nisha Abraham AU - Samantha Ali AU - Liliana Amaral-Almeida AU - John E. Cannon AU - Natalie Doughty AU - Choo Ng AU - Anie Ponnaberanam AU - Karen Sheares AU - Nicola Speed AU - Dolores Taboada AU - Mark Toshner AU - Steven Tsui AU - David P. Jenkins AU - Joanna Pepke-Zaba Y1 - 2020/01/01 UR - http://erj.ersjournals.com/content/early/2020/05/13/13993003.02096-2019.abstract N2 - 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 3 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 (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.Results Of 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.Conclusions PROs are markedly improved by PEA in patients with CTEPH, more so in those without clinically significant residual PH.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Newnham has nothing to disclose.Conflict of interest: Dr. Bunclark has nothing to disclose.Conflict of interest: Mrs Abraham has nothing to disclose.Conflict of interest: Mrs Ali has nothing to disclose.Conflict of interest: Dr. Almeida has nothing to disclose.Conflict of interest: Dr. Cannon reports grants from Actelion, during the conduct of the study; other from Actelion, other from GSK, outside the submitted work.Conflict of interest: Dr. Doughty has nothing to disclose.Conflict of interest: Dr. Ng has nothing to disclose.Conflict of interest: Dr. Ponnaberanam has nothing to disclose.Conflict of interest: Dr. Sheares reports educational support from Actelion, Bayer and GSK, personal fees from Actelion, outside the submitted work.Conflict of interest: Dr. Speed has nothing to disclose.Conflict of interest: Dr. Taboada reports other (Speaker honoraria and education/travel grants) from Actelion, Bayer, GlaxoSmithKline, Lilly, MDS, Pfizer, outside the submitted work.Conflict of interest: Dr. Toshner reports personal fees from Actelion/J&J, grants and personal fees from Bayer, personal fees from GSK, grants from Merck, outside the submitted work.Conflict of interest: Dr. TSUI has nothing to disclose.Conflict of interest: Dr. Jenkins reports grants and personal fees from Bayer, personal fees from Actelion, outside the submitted work.Conflict of interest: JPZ has participated in advisory board activities for Actelion, GSK, MSD given paid lectures for Actelion, MSD, my institution has received research, education grants from Actelion, MSD ER -