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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

Background— Chronic thromboembolic pulmonary hypertension results from incomplete resolution of pulmonary emboli. Pulmonary endarterectomy (PEA) is potentially curative, but residual pulmonary hypertension following surgery is common and its impact on long-term outcome is poorly understood. We wanted to identify factors correlated with poor long-term outcome after surgery and specifically define clinically relevant residual pulmonary hypertension post-PEA. Methods and Results— Eight hundred eighty consecutive patients (mean age, 57 years) underwent PEA for chronic thromboembolic pulmonary hypertension. Patients routinely underwent detailed reassessment with right heart catheterization and noninvasive testing at 3 to 6 months and annually thereafter with discharge if they were clinically stable at 3 to 5 years and did not require pulmonary vasodilator therapy. Cox regressions were used for survival (time-to-event) analyses. Overall survival was 86%, 84%, 79%, and 72% at 1, 3, 5, and 10 years for the whole cohort and 91% and 90% at 1 and 3 years for the recent half of the cohort. The majority of patient deaths after the perioperative period were not attributable to right ventricular failure (chronic thromboembolic pulmonary hypertension). At reassessment, a mean pulmonary artery pressure of ≥30 mm Hg correlated with the initiation of pulmonary vasodilator therapy post-PEA. A mean pulmonary artery pressure of ≥38 mm Hg and pulmonary vascular resistance ≥425 dynes·s −1 ·cm −5 at reassessment correlated with worse long-term survival. Conclusions— Our data confirm excellent long-term survival and maintenance of good functional status post-PEA. Hemodynamic assessment 3 to 6 months and 12 months post-PEA allows stratification of patients at higher risk of dying of chronic thromboembolic pulmonary hypertension and identifies a level of residual pulmonary hypertension that may guide the long-term management of patients postsurgery.

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 three 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 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.ResultsOut 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 pConclusionsPROs are markedly improved by PEA in patients with CTEPH, more so in those without clinically significant residual pulmonary hypertension.

Introduction Pulmonary endarterectomy (PEA) is the treatment of choice for Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and can be offered to selected patients with Chronic Thromboembolic Disease without pulmonary hypertension (CTED). Our aim was to map the pathway of UK patients referred to the National PEA MDT and their onwards management. Methods All consecutive individuals referred to the National PEA MDT at Papworth Hospital between June 2015 and May 2016 were included. Patients were divided into CTEPH/CTED groups and their pathways followed until July 2017. Results Of the 316 referrals to the PEA MDT there were 246 cases of CTEPH, 46 of CTED and 24 with alternate diagnoses. Age range of referrals was 22–88 years (mean 59 years). 51% were male. The majority of CTEPH cases had technically operable disease (n=200, 81%) with 130 proceeding to PEA (65%). 53% of all CTEPH cases were operated on (figure 1). Median time from first MDT discussion to PEA was 230 days with a median surgical waiting list time of 115 days. 11 patients required ongoing targeted therapy post-operatively. Of the 70 individuals with an operable disease distribution who did not proceed to PEA, 34 patients chose to decline surgery and 36 were rejected by the surgical team due to co-morbid conditions (n=28) or limited symptoms (n=8). Two patients with distal CTEPH underwent Balloon Pulmonary Angioplasty (BPA). Remaining patients were medically managed with targeted therapy. Of CTED cases, 28 (61%) had an operable disease distribution with 8 (29%) proceeding to PEA. 17% of total CTED referrals were operated on. Conclusion This patient pathway mapping is the first undertaken for any national CTEPH/CTED cohort. The majority of UK CTEPH cases suitable for PEA are operated on (65%). The duration of time between MDT referral and PEA is reflective of disease complexity and decision-making process. We observe a group of highly selected individuals with Chronic Thromboembolic Disease (CTED) without pulmonary hypertension who undergo PEA after careful consideration of their symptoms and operative risk.

Introduction The CAMPHOR (Cambridge Pulmonary Hypertension Outcome Review) score is an internationally validated patient reported outcome (PRO) measure for pulmonary hypertension, including chronic thromboembolic pulmonary hypertension (CTEPH). It assesses 3 areas: activity (score 0–30), symptoms (0–25) and quality of life (QoL) (0–25); with a higher score indicating a worse PRO. CTEPH frequently causes debilitating symptoms and functional impairment, which can be improved in selected patients with pulmonary endarterectomy (PEA). However, a subset will have residual pulmonary hypertension. We aim to assess PROs in patients with CTEPH undergoing PEA. Methods Consecutive CTEPH patients undergoing PEA from June 2006 to August 2016 at the UK National PEA centre, were included in this retrospective analysis. Patients are reviewed after PEA every 6–12 months for at least 5 years. CAMPHOR scores were recorded prospectively when patients attended hospital assessment and at each follow-up, ensuring high capture. Results 1151 patients underwent PEA during the study period. Of those, 937 (81%) had a CAMPHOR score recorded at baseline (pre-PEA) and 816 (77% of 1059 alive) at follow-up within a year of PEA (post-PEA). We confirmed significant improvements in 6 min walk distance and haemodynamics post-PEA (pre/post-PEA median ±IQR: 6 mwd 300±199 Metres/360±165; mPAP 45±15/25±13 mmHg; PVR 669±478/246±214 dynes.s.cm-5; CI 2.2±0.8/2.3±0.7 L/min/m2). The difference in median CAMPHOR scores pre- and post-PEA improved by 6,7 and 9 points for activity, QoL and symptoms respectively. The median difference for individuals having consecutive paired pre- and post-PEA scores also improved (median ±IQR: activity 4±7; QoL 4±8; symptoms 7±8). Patients were dichotomised into those with significant residual pulmonary hypertension (previously reported risk threshold of ≥30 mmHg , n=302) and those without (n=569). The improvement in CAMPHOR score was greater and more sustained in those without residual pulmonary hypertension (fsigure 1). Conclusion PROs relating to activity, QoL and symptoms improve after PEA in CTEPH when evaluated by CAMPHOR score. The improvement is sustained up to 5 years in those without residual pulmonary hypertension. Ongoing work will examine the utility of PROs in addition to traditional clinical outcome measures. Acknowledgements National Pulmonary Hypertension Centres UK and Ireland for referring patients considered for PEA.

Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) is a life threatening condition that historically has a poor outcome with supportive medical treatment. Pulmonary endarterectomy (PEA) is the treatment of choice and offers the only chance of cure. Data on the long term survival after PEA are limited. Method All patients who have undergone a PEA for CTEPH at Papworth hospital were included between January 1997 and November 2012. Patients who had a re-do operation were excluded. Pre- and post-operative data on haemodynamics, exercise capacity, functional class and targeted PAH therapies taken were obtained from our PH database and from other UK PH centres. The long-term survival of patients who returned for follow-up at 3 months post PEA was determined using the NHS spine summary care record tracking system. Overseas patients were censored when last seen. Results 880 patients underwent PEA over the 15 year period. The mean age was 57 (range 15–84) and 53% were male. The majority (89%) were in WHO functional class 3 or 4 prior to surgery with an average mean pulmonary artery pressure (mPAP) of 47 mmHg and PVR of 795 dynes. 65% of patients were taking at least 1 targeted therapy as a "bridge to surgery". Post surgery the majority of patients (86%) were in WHO functional class 1 or 2 at the 12 month follow-up with only 17% taking targeted therapy. There was a reduction in the average mPAP to 27 mmHg and PVR to 308 dynes by 12 months. The 10 year conditional survival post PEA of the first 314 patients from the cohort (Freed et al . J Thorac Cardiovasc Surg, 2011;141:383–7) was 74%. Conclusion The conditional survival of a subset of the cohort at 10 years was 74%. There was a significant functional and haemodynamic improvement in the majority of patients at 12 months post surgery. Only 17% of patients at 12 months post surgery were being treated with targeted therapy. Acknowledgements The authors would like to acknowledge the pulmonary hypertension centres in the UK. “ This research was supported by the National Institute for Health Research (NIHR) Cambridge Biomedical Research Centre ”.