RT Journal Article
SR Electronic
T1 Global burden of chronic thromboembolic pulmonary hypertension (CTEPH): An epidemiological analysis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P2326
VO 44
IS Suppl 58
A1 Henning Tiede
A1 Marius M. Hoeper
A1 Manuel Richter
A1 William Cacheris
A1 Barbara Hinzmann
A1 Eckhard Mayer
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P2326.abstract
AB IntroductionCTEPH is an orphan disease associated with significant morbidity and mortality, but data to quantify its global burden are limited. Here we attempt to estimate CTEPH global incidence.MethodsA systematic literature search was performed for CTEPH epidemiological data. As CTEPH can develop from acute pulmonary embolism (PE), searches included the proportion of PE patients (pts) developing CTEPH, and the proportion of CTEPH pts with unknown etiology. Where multiple values were found for search parameters, a weighted average was used. Data were used to estimate CTEPH incidence in various countries.ResultsThe search yielded 151 publications, 21 of which were selected. The age and sex-specific incidence of acute PE produced crude rates of 60–100 cases/100000 in Europe and the US, and 4–6/100000 in Japan and China. The rate of CTEPH in pts with previous PE was estimated at 4% in Europe and the US, and 14% in Japan. Surprisingly, 40%–60% of CTEPH pts in Europe and the US, and 67% in Japan had no history of acute PE. The projected incidence of CTEPH for 2014 in Europe, the US, Japan, and China is 66000 cases (Table 1). Based on PE rates and population projections, CTEPH incidence will increase to >75000 cases in 2025.View this table:Estimated CTEPH incidenceConclusionsThis study highlights a possible increase in CTEPH burden and the need to raise disease awareness. Estimated incidence is useful to understand CTEPH global epidemiology, but there is a lack of epidemiological and individual country data. It will be important to obtain more robust data in the future.