Epidemiological data for chronic thromboembolic pulmonary hypertension (CTEPH) are limited and there are conflicting reports regarding its pathogenesis.A literature review was conducted to identify CTEPH epidemiological data up to June 2014. Data were analysed to provide estimates of the incidence of CTEPH in the USA, Europe and Japan. An epidemiological projection model derived country-specific estimates of future incidence and diagnosis rates of CTEPH.Overall, 25 publications and 14 databases provided quantitative epidemiological data. In the USA and Europe, the crude annual incidence of diagnosed pulmonary embolism and crude annual full (i.e. diagnosed and undiagnosed) incidence of CTEPH were 66-104 and 3-5 cases per 100 000 population, respectively, while in Japan these rates were lower at 6.7 and 1.9 per 100 000 population, respectively. In 2013, 7-29% of CTEPH cases in Europe and the USA were diagnosed, and the majority of patients were in New York Heart Association functional class III/IV at diagnosis. The projection model indicated that incidence of CTEPH will continue to increase over the next decade.These data suggest that CTEPH is underdiagnosed and undertreated, and there is an urgent need to increase awareness of CTEPH. High-quality epidemiological studies are required to increase understanding of CTEPH.

ObjectivesTo better understand the patient's perspective of pulmonary hypertension (PH), including the impact of living with PH, disease management and treatment.DesignThis qualitative ethnographic study collected observational video footage, supplemented by field notes and patient diaries to assess the impact of PH on the patient's life.SettingPatients were observed and filmed in their home for up to 6 h, capturing the environment, interactions and activities of everyday life.ParticipantsPatients with pulmonary arterial hypertension (PAH) or chronic thromboembolic PH who were receiving PAH-specific medication were recruited through healthcare professionals (HCPs) and patient associations in seven countries across four continents. Sampling was purposive and subgroup analysis was not intended.ResultsOverall, 39 patients with PH were enrolled. Many patients had a poor understanding of PH and found their ‘invisible’ disease difficult to explain to others. An important finding was the secrecy surrounding PH. Feelings of insecurity and isolation were regularly reported, and many patients admitted to hiding their symptoms. The marked improvement in symptoms after therapy initiation made assessment of disease progression more difficult as patients compared their quality of life (QoL) against pretreatment levels. Extensive planning and adherence to daily routines were required in patients’ everyday life.ConclusionsEthnography was used for the first time, in several countries, to evaluate the patient's perception of living with PH. This approach revealed key findings that would not typically be uncovered using other qualitative techniques, including the secrecy surrounding PH, the difficulties in describing the disease and the challenges in assessing disease progression. A more tailored dissemination of information from HCPs and development of a simple and understandable PH definition may be beneficial in alleviating the secrecy reported by patients. A greater appreciation of how patients perceive their disease and QoL has the potential to improve PH management.

Abstract:We conducted an international study to evaluate practices in the diagnosis and management of patients with chronic thromboembolic pulmonary hypertension (CTEPH) globally across different regions. Between August and October 2012, CTEPH-treating physicians completed a 15-minute online questionnaire and provided patient record data for their 2-5 most recent patients with CTEPH. Overall, 496 physicians (Europe: 260; United States: 152; Argentina: 52; Japan: 32) completed the questionnaire and provided patient record data for 1,748 patients. The proportion of physicians who described themselves as working in or affiliated with a specialized pulmonary hypertension (PH) center ranged from 38% in France and Italy to 83% in the United States. A large proportion of patients did not undergo ventilation/perfusion scanning (46%-67%) or right heart catheterization (24%-57%) for the diagnosis of CTEPH. Referral rates for pulmonary endarterectomy evaluation ranged from 25% in Japan to 44% in Europe, with higher referral rates in PH centers; the main reasons for lack of referral were that surgery was not considered unless medical treatment was failing and patient refusal. Other variations in management included greater use of phosphodiesterase 5 inhibitors in the United States than in Europe and Japan and greater use of combination treatment in the United States than in Europe. Physicians' perceptions of their treatment strategy were generally consistent with patient record data. Results from this study, which includes a global aspect of CTEPH care, demonstrate not only regional differences in CTEPH management but, more importantly, considerable nonadherence to the diagnosis and treatment guidelines for CTEPH, even in PH centers.Keywords: chronic thromboembolic pulmonary hypertension, pulmonary arterial hypertension-specific therapies, diagnosis, clinical practice, guidelines. Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease of obstructive pulmonary artery remodeling as a consequence of major vessel thromboembolism. 1 A ventilation/perfusion (V/Q) scan is recommended in the workup of all patients with pulmonary hypertension (PH) to screen for CTEPH. 1 Diagnosis of CTEPH is challenging for several reasons, including nonspecific presenting symptoms, which may occur late in the progression of the disease, and a lack of prior pulmonary embolism in a high percentage of patients. 2 Once CTEPH is diagnosed, pulmonary endarterectomy (PEA) is the gold-standard treatment for these patients and is potentially curative. 1,3 However, between 20% and 40% of these patients are considered to have inoperable CTEPH, 4 and approximately 30% may have residual PH after PEA (defined as mean pulmonary artery pressure of ≥30 mmHg at 3-month follow-up after surgery). 5 Balloon pulmonary angioplasty (BPA) is an emerging treatment option for patients with inoperable or persistent CTEPH. 3 However, the role of BPA in CTEPH has not been investigated in randomized trials, and further studies are warranted; current guidelines state ...

ObjectiveTo gain insight into clinical practice regarding referral, early diagnosis and other aspects of the management of patients with dcSSc in Europe and the USA. MethodsSemi-structured interviews were conducted with 84 rheumatologists (or internal medicine physicians) and 40 dermatologists in different countries (the UK, France, Germany, Italy, Spain and the USA). Physicians were asked to identify key steps in the patient pathway relating to patient presentation, diagnosis and referral, in addition to other treatment and follow-up processes.ResultsThe interviewed physicians reported that late presentation with dcSSc was common, with some patients presenting to primary care physicians after symptoms had persisted for up to 1 year. Awareness of dcSSc is reported to vary widely among primary care physicians. Final diagnosis, generally following guideline-based recommendations, was by rheumatologists in most cases (or internal medicine physicians in France) and they remained responsible for global patient management, with lesser involvement in diagnosis and management by dermatologists. Specialist centres were not well defined and did not exist in all countries.ConclusionPatients and primary healthcare providers can be unaware of the symptoms of dcSSc, therefore presentation and referral to specialist care are often late. Thus, improved awareness among patients and primary care physicians is necessary to facilitate earlier referral and diagnosis. Once referred, more consistent use of the modified Rodnan skin score at diagnosis and follow-up may help to monitor disease progression. Furthermore, establishing specialist centres may help to promote such changes and improve patient care.

Abstract:We conducted an international study to evaluate practices in the diagnosis and management of pulmonary arterial hypertension (PAH) globally across different geographic regions. Between July and October 2012, PAH-treating physicians completed a 15-minute online questionnaire and provided patient record data for their 3 or 5 most recent patients with PAH. Overall, 560 physicians (Europe: 278; United States: 160; Argentina: 53; Japan: 69) completed the questionnaire and provided data for 2,618 patients. The proportion of physicians who described themselves as working in or affiliated with a specialized pulmonary hypertension center ranged from 13% in Argentina to 74% in the United States. At the time of diagnosis, patients' New York Heart Association functional class differed significantly between regions. At the time of last assessment, functional class had improved overall, and differences between regions had largely disappeared. A large proportion of patients did not undergo right heart catheterization for the diagnosis of PAH (Europe: 7%-21%; United States: 21%; Japan: 19%; Argentina: 51%). Variations in management included greater use of phosphodiesterase 5 inhibitors in the United States than in Europe and Japan and greater use of triple or greater combination therapy in Japan than in other regions. Results from this study, which includes a global aspect of PAH care, demonstrate that there are significant differences in PAH management between regions and low adherence to guidelines recommending right heart catheterization for the diagnosis of PAH.Keywords: pulmonary arterial hypertension, pulmonary arterial hypertension-specific therapies, diagnosis, clinical practice, guidelines. Pulmonary arterial hypertension (PAH; World Health Organization group I) is defined by a mean pulmonary artery pressure of ≥25 mmHg and increased pulmonary vascular resistance of >3 Wood units with a normal pulmonary capillary wedge pressure. 1,2 PAH is a progressive disease that can ultimately lead to right heart failure and death. 1,3 Despite advances in current therapies for PAH, there is still significant unmet medical need, especially in areas of the world where access to diagnostic and therapeutic options is limited. The mortality of patients with PAH remains high even in well-developed countries: 15% at 1 year and 32% at 3 years. 4 The therapeutic approach to PAH is evolving; multiple classes of agents are available, and physicians from both expert pulmonary hypertension (PH) centers and the community treat patients with PAH. 5,6 Data from various registries suggest that approaches to the diagnosis and treatment of PAH may differ between countries. [7][8][9][10][11] In this international study, we sought to assess differences in the diagnosis and management of patients with PAH across different countries and regions worldwide by conducting a large, physicianbased study using a quantitative online questionnaire. The objectives of the study were (1) to assess the diagnosis and management of PAH in different countries a...