Table 1.
Study details and epidemiology estimates from identified studies investigating PAH epidemiology in adults.
| Study classification | Study description | Country | Time period range | Number of participants | Publication (study acronym) | Annual incidence (ppm) | Prevalence (ppm) |
|---|---|---|---|---|---|---|---|
| National systematic registry | National systematic registry | UK | 2018–2019 | NR | NHS Digital, 201916,a | – | 54.7b |
| Sweden | 2008–2019 | 1034 | Kjellström et al., 2020 (SPAHR)17,a | 5.8b | 47.6b | ||
| UK (Scotland) | 1997–2006 | NR | Peacock et al., 200725,a | 7.6 | 26 | ||
| Czech Republic | 2000–2007 | 191 | Jansa et al., 201441,a | 10.7 | 22.4 | ||
| Latvia | 2007–2016 | 130 | Skride et al., 201820,a | 13.7 | 45.7 | ||
| Non-systematic | National, non-systematic | Portugal | 2008–2010 | 46 | Baptista et al., 201344,a | 1.5 | – |
| South Korea | 2008–2011 | 297 | Chung et al., 2015 (KORPAH)26 | 1.9 | – | ||
| France | 2002–2003 | 674 | Humbert et al., 200622,a | 2.4 | 15 | ||
| Switzerland | 1999–2004 | 152 | Tueller et al., 200823 | 3.5 | 15.5 | ||
| Spain | 2007–2008 | 866 | Escribano-Subias et al., 2012 (REHAP)33,a | 3.7 | 16 | ||
| Germany | 2007–2014 | 1752 | Hoeper et al., 2016 (COMPERA)21,a | 3.9 | 25.9 | ||
| Poland | 2018 | 970 | Kopeć et al., 2020 (BNP-PL)19,a | 5.2 | 30.8 | ||
| Non-national (multi-centre) | USA | 2006–2007 | 2967 | Frost et al., 2011 (REVEAL)5,a,c | 2.3 | 12.4 | |
| Claims/administrative databases | Claims (Medicare) | USA | 1999–2007 | 215 | Kirson et al., 201128 | – | 30.4b |
| Claims (PHIC) | Colombia | 2015 | 18 | Miranda-Machado et al., 201947,a | – | 28 | |
| Claims (HIRA) | South Korea | 2008–2016 | 1307 | Song et al., 201827 | 4.8 | 20.2 | |
| National hospitalization database (SMR) | UK (Scotland) | 1986–2001 | 374 | Peacock et al., 200725 | 7.1 | 52 | |
| Administrative database (ICES) | Canada | 1993–2012 | 6705 | Wijeratne et al., 201824 | 32 | 268 | |
| Clinical databases | Single-centre study | UK | 2001–2010 | 598 | Hurdman et al., 2012 (ASPIRE)48,a | 6.1 | – |
| Single-centre study | Israel | 1998–2005 | 84 | Fruchter and Yigla, 200849 | 7.1b | – | |
| Single-centre study | USA | 2016 | 154 | Dubroff et al., 202050,a | 14 | 93 |
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.
aPAH definition included mPAP >/≥ 25 mmHg at rest (± or >30 mmHg on exercise) and PAWP </≤ 15 mmHg, as assessed by RHC.
bEstimates are derived from the publication using the method outlined in Table 5.
cREVEAL registry included an expanded criterion for patients with PAWP ≤18 mmHg.
ASPIRE: Assessing the spectrum of pulmonary hypertension identified at a REferral centre; COMPERA: Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension; HIRA: Health Insurance Review and Assessment Service; ICES: Institute for Clinical Evaluative Sciences; KORPAH: Korean Registry of Pulmonary Arterial Hypertension; NHS: National Health Service; NR: not reported; PAWP: pulmonary arterial wedge pressure; ppm: patients per million; REHAP: Spanish Registry of Pulmonary Arterial Hypertension; REVEAL: The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management; RHC: right heart catheterisation; SMR: Scottish Morbidity Record scheme; SPAHR: Swedish Pulmonary Arterial Hypertension Registry; BNP-PL: Polish Registry of Pulmonary Hypertension.