TY - JOUR T1 - Epidemiology of chronic thromboembolic pulmonary hypertension (CTEPH) in the Czech Republic JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2020.1543 VL - 56 IS - suppl 64 SP - 1543 AU - Pavel Jansa AU - David Ambrož AU - Matyáš Kuhn AU - Vladimír Dytrych AU - Aleš Linhart AU - Jaroslav Lindner AU - Audrey Muller Y1 - 2020/09/07 UR - http://erj.ersjournals.com/content/56/suppl_64/1543.abstract N2 - CTEPH is a severe progressive pulmonary vascular disease for which multimodal treatment became recently available. We report the epidemiology and survival of all adult CTEPH patients diagnosed between 2003–2016 in the Czech Republic, where diagnosis and management are centralised in a single expert centre.During the study period, the main treatment option was pulmonary endarterectomy (PEA), the gold standard treatment for CTEPH. Medical treatment was limited to riociguat since 2015, and to participation in clinical trials with other drugs, as off-label use is not allowed. Balloon pulmonary angioplasty (BPA) was introduced in 2016. For incidence/prevalence rates calculation, adult general population data were extracted from the Institute of Health Information and Statistics. Survival from diagnosis was calculated using Kaplan-Meier estimates.Characteristics of patients (N=453) at diagnosis were: median age of 65.2 years, 55% male, 92% NYHA functional class III–IV, and median 6-minute walk test of 336 m. Median time from diagnosis to last follow-up was 6.1 years. Incidence (2006–2016) was 4.47/1,000,000 person-years and prevalence was 37.43 (2016). 236 (52%) patients benefitted from PEA. Of the not operated patients (n=217), 59% (n=129) were technically inoperable (distal disease) and 41% (n=88) were medically inoperable (comorbidities/refusal). 5-year survival probability (95% confidence interval) was: 72.3% (67.8; 76.3) for overall, 82.2% (76.5; 86.5) for operated, 65.3% (56.0; 73.2) for technically inoperable and 55.2% (43.6; 65.4) for medically inoperable.These data indicate an unmet need in inoperable patients, during a time period with limited use of BPA/medical treatment options.FootnotesCite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 1543.This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -