Table 3.
Study details and epidemiology estimates from identified studies investigating CTEPH epidemiology in adults.
| Study classification | Study description | Country | Time period | Number of participants | Publication (study acronym) | Annual incidence (ppm) | Prevalence (ppm) |
|---|---|---|---|---|---|---|---|
| National systematic registry | National systematic registry | Sweden | 2008–2019 | 417 | Kjellström et al., 2020 (SPAHR)17 | 3.1a | 25.8a |
| Latvia | 2007–2016 | 44 | Skride et al., 2018 20 | 5.1 | 15.7 | ||
| UK | 2018–2019 | 2492 | NHS Digital, 201916 | 6.0a | 38.4a | ||
| Non-systematicregistry | National, non-systematic | Slovakia | 1998–2014 | 81 | Bohacekova et al., 201655 | – | 18 |
| Portugal | 2008–2010 | 33 | Baptista et al., 201344 | 1.1 | – | ||
| Spain | 2007–2018 | 1019 | Martínez-Santos et al., 2019 (REHAP)56 | 1.7 | 22.5 | ||
| Germany | 2016 | 392 | Kramm et al., 2018 (COMPERA)57 | 5.7 | – | ||
| Claims/administrative database | Claims | USA | 1999–2007 | 431 | Kirson et al., 201128 | – | 39.4a |
| Administrative database (PMSI) | France | 2009-2015 | 3138 | Cottin et al., 201958 | – | 47 | |
| Administrative database (ICES) | Canada | 1993–2012 | 4360 | Wijeratne et al., 201824 | 39a | 144 | |
| Clinical database | Multi-centre | Spain | 1998–2017 | 42 | Llanos-González et al., 201959 | 0.9 | 14.5 |
| Single-centre study | Israel | 1998–2005 | 16 | Fruchter et al., 200849 | 1.3 | – | |
| Single centre study | UK | 2001–2006 | 105 | Condliffe et al., 200860 | 1.8 | – | |
| Single-centre study | UK | 2001–2010 | 242 | Hurdman et al., 2012 (ASPIRE)48 | 3.7 | – | |
| Single-centre study | Thailand | 2012–2016 | 20 | Puengpapat and Pirompanich, 201861 | 37.8 | – |
Notes: Studies are ordered by study design and then in ascending order of incidence estimate. Estimates are rounded to one decimal place, except where only integers were published.
aEstimates are derived from the publication using the method outlined in Table 5.
ASPIRE: assessing the spectrum of pulmonary hypertension identified at a REferral centre; COMPERA: Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension; ICES: Institute for Clinical Evaluative Sciences; NHS: National Health Service; PMSI: French exhaustive hospital discharge database; ppm: patients per million; REHAP: Spanish Registry of Pulmonary Arterial Hypertension; SPAHR: Swedish Pulmonary Arterial Hypertension Registry.