RT Journal Article SR Electronic T1 The impact of patient choice on survival in chronic thromboembolic pulmonary hypertension JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 1800589 DO 10.1183/13993003.00589-2018 A1 SR Quadery A1 AJ Swift A1 C Billings A1 AAR Thompson A1 CA Elliot A1 J Hurdman A1 A Charalampopoulos A1 I Sabroe A1 I Armstrong A1 N Hamilton A1 P Sephton A1 S Garrod A1 J Pepke-Zaba A1 DP Jenkins A1 N Screaton A1 AM Rothman A1 A Lawrie A1 T Cleveland A1 S Thomas A1 S Rajaram A1 C Hill A1 C Davies A1 CS Johns A1 JM Wild A1 R Condliffe A1 DG Kiely YR 2018 UL http://erj.ersjournals.com/content/early/2018/06/14/13993003.00589-2018.abstract AB Background Pulmonary endarterectomy (PEA) is the gold standard treatment for operable chronic thromboembolic pulmonary hypertension (CTEPH). However, a proportion of patients with operable disease decline surgery. There are currently no published data on this patient group. The aim of this study was to identify outcomes and prognostic factors in a large cohort of consecutive patients with CTEPH.Methods Data were collected for consecutive, treatment-naïve CTEPH patients between 2001–2014 identified from the ASPIRE registry.Results Of 550 CTEPH patients (age 63±15 years, follow-up 4±3 years), 49 percent underwent surgery, 32% had technically operable disease and did not undergo surgery (including patient choice n=72, unfit for surgery n=63) and 19% had inoperable disease due to disease distribution. Five-year-survival was superior in patients undergoing PEA (83%) versus technically operable disease who did not undergo surgery (53%) and inoperable due to disease distribution (59%), p<0.001. Survival was superior in patients following PEA compared to those offered but declining surgery (55%), p<0.001. In patients offered PEA, independent prognostic factors included mixed venous oxygen saturation, gas transfer and patient decision to proceed to surgery.Conclusions Outcomes in CTEPH following PEA are excellent and superior to patients declining surgery and strongly favour consideration of a surgical intervention in eligible patients.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. Swift has nothing to disclose.Conflict of interest: Dr. Thompson reports non-financial support (travel grants) from Actelion Pharmaceuticals Ltd, outside the submitted work.Conflict of interest: Dr. Elliot reports personal fees from Actelion Pharmaceuticals, personal fees from Glaxo SmithKline, grants from Pfizer, grants from Actelion Pharmaceuticals, grants from Bayer, grants from Bayer, grants from Actelion Pharmaceuticals, personal fees from Bayer, outside the submitted work; .Conflict of interest: JH was part funded as a clinical research fellow by an unrestricted educational grant from Actelion and has received funding to attend conferences from Actelion, GSK and Pfizer.Conflict of interest: I have received honoraria for lecturing and taking part in advisory boards as well as support to attend scientific events/congresses by Actelion, GSK, Servier and MSD.Conflict of interest: Dr. Sabroe has nothing to disclose.Conflict of interest: Dr. Armstrong has nothing to disclose.Conflict of interest: Dr. Hamilton I have and continue to receive honoraria for participation in advisory boards and educational meetings and have and continue to receive funding to attend educational meetings from a number of pharmaceutical companies including Actelion, Bayer, GSK and MSD. These companies manufacture drug therapies for a variety of indications but include my areas of clinical interest in pulmonary hypertension.Conflict of interest: Dr. Sephton has nothing to disclose.Conflict of interest: S. GarrodConflict of interest: JPZ or her institution, have received research, educational grants, and speaker's honoraria from Actelion, Bayer Pharma AG, Merck and GSKConflict of interest: Dr. Jenkins reports personal fees from Bayer, personal fees from Actelion, outside the submitted work.Conflict of interest: Dr. Screaton has nothing to disclose.Conflict of interest: Dr. Rothman has nothing to disclose.Conflict of interest: Dr. Lawrie reports grants from British Heart Foundation, grants from Medical Research Council UK, grants from Actelion Pharmaceuticals, grants from GSK, other from Actelion Pharmaceuticals, other from Actelion Pharmaceuticals, outside the submitted work.Conflict of interest: Dr. Cleveland has nothing to disclose.Conflict of interest: Dr. Thomas has nothing to disclose.Conflict of interest: Dr. Rajaram has nothing to disclose.Conflict of interest: Dr. Hill has nothing to disclose.Conflict of interest: Dr. Davies has nothing to disclose.Conflict of interest: Dr. Johns has nothing to disclose.Conflict of interest: Dr. Wild has nothing to disclose.Conflict of interest: Dr. Condliffe reports personal fees from Actelion, personal fees from Bayer, personal fees from GSK, outside the submitted work.Conflict of interest: Dr. Kiely reports grants, personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Bayer, grants, personal fees and non-financial support from GSK, personal fees and non-financial support from MSD, outside the submitted work.Conflict of interest: Dr. Billing has nothing to disclose.Conflict of interest: Nurse Garrad has nothing to disclose.Conflict of interest: Dr. Quadery has nothing to disclose.