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Rationale: The management of chronic thromboembolic pulmonary hypertension (CTEPH) has changed over recent years with the growth of pulmonary endarterectomy surgery and the availability of disease-modifying therapies.Objectives: To investigate the prognosis of CTEPH in a national setting during recent years.Methods: All incident cases diagnosed in one of the five pulmonary hypertension centers in the United Kingdom between January 2001 and June 2006 were identified prospectively. Information regarding baseline characteristics, treatment, and follow-up was subsequently collected from hospital records.Measurements and Main Results: A total of 469 patients received a diagnosis, of whom 148 (32%) had distal, nonsurgical disease. One- and three-year survival from diagnosis was 82 and 70% for patients with nonsurgical disease and 88 and 76% for those treated surgically (P = 0.023). Initial functional improvement in patients with nonsurgical disease was noted but did not persist at 2 years. Significant functional and hemodynamic improvements were seen in surgically treated patients with an increase in six-minute-walk distance of 105 m (P < 0.001) at 3 months. Five-year survival from surgery in the 35% of patients who survived to 3 months but had persistent pulmonary hypertension was 94%.Conclusions: The prognosis in nonsurgical disease has improved. We have confirmed the previously described good outcome in surgically treated disease. However, we have also demonstrated that the long-term prognosis for patients who have persistent pulmonary hypertension at 3 months after surgery is good. The observed improvements in outcome during the modern treatment era reinforce the importance of identifying patients with this increasingly treatable condition. [ABSTRACT FROM AUTHOR]

Introduction and Objectives Markers of immune activation and inflammation are raised in pulmonary arterial hypertension; the degree of elevation of levels of pro-inflammatory cytokines in idiopathic pulmonary arterial hypertension has recently been shown to predict long term survival (Soon et al, Circulation, 2010). Much less is known of the role of cytokines in disease monitoring. We assessed levels of serum cytokines in patients at initial assessment prior to commencement of targeted therapy for pulmonary hypertension, and when the patient returned to the hospital for follow-up. We also examined if changes in serum cytokines correlated with changes in 6-min walk test (6MWT). Methods 19 patients with a diagnosis of Group I PAH or Group IV chronic thromboembolic pulmonary hypertension in a distribution inaccessible to surgery were included. Patients with inflammatory conditions other than an associated connective tissue disease or a musculoskeletal condition that would interfere with the 6MWT were excluded. 11 healthy volunteers acted as a control group. Serum levels of interleukin (IL) 1β, 2, 4, 6, 8, 10, 12p70 and tumour necrosis factor α (TNFα) were measured using a multiplex analyser. A Wilcoxon matched pairs test was used to compare differences between baseline and follow-up samples. Results Patient and control demographics are presented in the Abstract P3 table 1. Levels of IL 2,6,8,10 and TNFα were elevated in the patient group compared to controls. Average time to repeat patient sampling was 4.7±1.8 months. There were no significant differences between the levels of any cytokines between baseline and follow-up in the patients and no overall correlation existed between change in 6MWT and change in any cytokine. When patients who had improved their 6MWT on follow-up were analysed as a separate group, there was a significant (p=0.0068) drop in IL6 levels on follow-up. Conclusions Several cytokines were elevated in these patients with pulmonary hypertension. The role of cytokines in disease monitoring requires further study and there seems to be little relation with serum cytokines, except IL6, and a change in a patient's clinical state as measured by 6MWT. Replication of results from this pilot study and examination of longitudinal trends in cytokine levels are warranted. [ABSTRACT FROM AUTHOR]

Introduction The NIH survival equation was developed in the 1980s and used in many clinical trials to show survival benefit. Since then, significant advances had been made in the diagnosis and management of pulmonary arterial hypertension (PAH). Several new equations have been developed to better predict survival in PAH. All these survival equations were derived using prognostic factors identified from a mixed incident and prevalent PAH cohort. However, prevalent patients have better survival compared to incident patients. Objectives To determine applicability of currently available survival equations in incident idiopathic (IPAH), heritable and anorexigen-associated PAH. Methods Retrospective observational study of all incident cases of IPAH, heritable and anorexigen-associated PAH diagnosed in the UK and Ireland between 1st January 2001 and 31st December 2009. Observed survival in the UK and Ireland was estimated using life table analysis and compared with predicted survival in the same patients calculated using the NIH, French, Pulmonary Hypertension Connection (PHC) and REVEAL Registry equations. Results Total of 646 patients were diagnosed over the study period. Observed vs predicted survival were compared [Abstract S72 figure 1]. Conclusion Survival equations derived from other mixed incident and prevalent pulmonary hypertension populations may not accurately predict survival of incident pulmonary arterial hypertension from the UK and Ireland. Differences in baseline characteristics, treatment practice and time period between our patients and survival equations derivation populations need to be taken into account when applying these equations in daily clinical practice. [ABSTRACT FROM AUTHOR]

Introduction and Objectives The age of patients with idiopathic pulmonary arterial hypertension (IPAH) has increased since the NIH registry. It is postulated that older IPAH patients may have a different disease phenotype compared to their younger counterparts. Methods Retrospective observational study of all consecutive incident cases of IPAH, heritable and anorexigen-associated pulmonary arterial hypertension diagnosed in all eight pulmonary hypertension centres in the UK and Ireland between 1st January 2001 and 31st December 2009. Patients were divided into younger and older subgroups by the median age (57 years). Results Of 646 incident cases (mean age=54), 22% were over the age of 70 and 3% over age 80. Younger patients had higher % female (71% vs 56%), shorter duration of symptoms (median 15 months vs 21 months), better functional class (18% in functional class I/II vs 10%), exercise capacity (6-minute walk distance 325 m vs 217 m) and higher % predicted DLCO (65% vs 47%) compared to older patients. Older patients were more likely to present with peripheral oedema (41% vs 28%) whereas younger patients were more likely to complain of syncope (30% vs 10%), presyncope (14% vs 7%) and fatigue (15% vs 9%) at the time of diagnosis. Younger patients had higher mean pulmonary artery pressure (56 mm Hg vs 49 mm Hg) and pulmonary vascular resistance index (24 WU.m² vs 21 WU.m²) but lower wedge pressure (9 mm Hg vs 10 mm Hg) compared to older patients. Patients in the highest age quartile had the worst survival [Abstract S71 figure 1]. Conclusion Older incident IPAH patients appear to have a different disease phenotype compared to younger patients. IPAH is no longer a disease that affect predominantly young female only. [ABSTRACT FROM AUTHOR]

Introduction Pulmonary endarterectomy (PEA) is the treatment of choice for patients with proximal chronic thromboembolic pulmonary hypertension (CTEPH). The UK has a single centre performing this operation and the program became nationally funded since 2000. Patients are referred from seven specialist pulmonary hypertension centres. Method All 625 patients treated with PEA from 2000 to 2010 were mapped according to their home postcode at the time of PEA surgery. Primary care trusts (PCT's) were assigned from these home postcodes. The incidence rate of each PCT and overall incidence were analysed. Map info software was used to generate the referral maps. Results The new patient incidence ranges from no referrals in 123 PCT's in 2000-2002 to 90 in 2008-2010. The most recent period shows highest referral rates for PEA. From our PEA data mapping analysis we have calculated that the incidence of operated patients was 0.4 million population in 2000 (n=22) and 2 per million population in 2010 (n=122). Conclusion There has been a fivefold increase in PEA activity in the UK over the last decade. The analysis of our data are limited to surgical cases. The current incidence of PEA in the UK is already higher than historical estimation of the incidence of all CTEPH (0.1-0.5/million, Fedullo, N Engl J Med 2001). Since 30% of patients with CTEPH have distal disease distribution and some patients with proximal CTEPH do not proceed to surgery due to choice or comorbidities, the overall incidence of CTEPH is likely to be significantly higher than 2 per million/year and higher than previously suspected. [ABSTRACT FROM AUTHOR]

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is defined by a mean pulmonary artery pressure (mPAP) =25 mm Hg at right heart catheterisation in patients with multiple chronic/organised occlusive thrombi in the pulmonary arteries. PEA is potentially curable and is the treatment of choice for patients with CTEPH. There is a small group of patients with CTED and exertional dyspnoea but with a resting mPAP that does not fulfil criteria for PH in whom PEA may be considered. The outcome of PEA in these patients has not been previously assessed. Objective To assess the post PEA functional and haemodynamic outcomes in patients with symptomatic CTED and borderline PH. Methods Retrospective data were collected on patients with baseline mPAP of =25 mm Hg who underwent PEA at the UK and Ireland national referral centre between 2002 and 2010. Patients were reassessed 3 and 12 months after surgery. Right heart catheterisation was performed at baseline and 3 months post PEA. Results 558 patients underwent PEA at Papworth Hospital between 2002 and 2010. From those, 16 had symptomatic operable CTED and mPAP of =25 mm Hg. Mean age was 45±17 and 69% were female. All survived surgery and are currently alive at follow-up. The median length of stay was 13 days (IQR 6). Results are displayed in the Abstract S22 table 1 as mean ± SD. WHO class data are expressed as percentages. CAMPHOR (Cambridge Pulmonary Hypertension Outcome Review) is a disease specific quality of life questionnaire and scores are expressed as median. Conclusion In this small series of patients with CTED and borderline PH, there was a significant symptomatic, haemodynamic and functional benefit from PEA at 1-year. Further research is required to assess the prognostic benefit in this population. We would like to acknowledge the national pulmonary hypertension centres in the UK and Ireland, and support by the Cambridge NIHR Comprehensive Biomedical Research Centre. [ABSTRACT FROM AUTHOR]

The management of CTEPH has evolved due to advances in diagnosis, treatment, and the adoption of best practice guidelines. However, there is limited information regarding contemporary real-world clinical care. The aim of this survey was to collect clinical practice data from hospital-based medical specialists and to identify unmet needs in CTEPH. CLARITY was developed by an independent committee of international CTEPH experts using the Delphi method. Questions were designed to gather quantitative and qualitative insights on the awareness, diagnosis, and treatment of CTEPH. Twenty-one international, regional, and national scientific societies and other medical organizations have supported the web-survey distribution. Responses were collected from 09.10.2021 to 05.01.2022. 416 questionnaires were collected, 353 of which were suitable to be analyzed. Respondents came from Europe (44%), Asia-Pacific (32%), Latin America (11%), US / Canada (11%), and the Middle East / Africa (2%). Most respondents specialized in pulmonology (44%) or cardiology (41%) and had over 15 years of experience (56%). Around 30% were not working in a pulmonary hypertension or CTEPH expert center and among them, only 30% were affiliated to such a center. The most commonly reported barriers to CTEPH recognition and diagnosis, operability assessment, and treatment by pulmonary endarterectomy (PEA) / balloon pulmonary angioplasty (BPA) were lack of disease awareness and structured pulmonary embolism follow-up, delayed referral, lack of standardized operability criteria and multidisciplinary team/reference center, limited access to PEA / BPA, and insufficient expertise in performing PEA / BPA, respectively (Table). The survey provided global insights on the contemporary management of CTEPH and an understanding of key barriers to optimal care. It may serve as a basis to call for action to improve CTEPH detection and management. [ABSTRACT FROM AUTHOR]