Table 4

Incidence and survival of PAH in Ireland and other European countries

Country	Ireland	UK14 (Scotland)	UK17 (ASPIRE)	France22 33	Spain23	Sweden12 16 (SPAHR)	Czech15	Germany24 (Giessen)	Germany25 (COMPERA)	Latvia13	Denmark26	Portugal34
Subjects with PAH, n	163	374	1344	674	866	457	191	685	263	130	134	46
Recruitment period	2010–2020	1997–2006	2001–2010	2002–2003	1998–2008	2000–2014	2000–2007	2008–2011	2014	2007–2016	2000–2012	2008–2010
Age (years), mean±SD	56±15	52±13*	59±17	50±15	45±17	67†	52±17	51±16	61±18	65†	50±21	43±16
Female, %	77%	70%		65.3%	71%	64%	65%	65%	67%	73%	58.2%	65.2%
Annual incidence
PAH	3.11	7.6	6.1	2.4	3.7	-	10.7	-	3.9	13.7	-	1.5
IPAH	0.63	2.6	2.1	-	1.2	5‡	6.2‡	-	2.4	7.6	-	-
CTD-PAH	1.53	2.8	2.4	-	-	-	-	-	1.0	-	-	-
Survival PAH, %
1 year	89	-	88	87	87	85	89	88.2	-	88	86.4	93.5
3 year	75	-	68	67	75	71	74	72.2	-	73.3	72.9	-
5 year	65	-	-	-	65	59	-	59.4	-	58.1	65.4	-
Survival IPAH, %
1 year	100	-	-	89§	89	-	85‡	89.7	-	-	-	-
3 year	100	-	63	69§	77	-	62‡	76.2	-	-	-	-
5 year	95	-	-	-	68	-	-	65.3	-	-	-	-
Survival CTD-PAH, %
1 year	83	-	-	-	-	-	-	85.3	-	-	-	-
3 year	62	-	54	-	-	-	-	65.6	-	-	-	-
5 year	47	-	-	-	-	-	-	50.9	-	-	-	-

The annual incidence is calculated per million population. Age is displayed as mean and SD unless otherwise specified.
*Female mean age (SD).
†Median.
‡Incidence reflects IPAH and HPAH combined.
§Incidence reflects IPAH, HPAH and Anorexigen-associated PAH.
CTD-PAH, connective tissue disease associated PAH; IPAH, idiopathic PAH; PAH, pulmonary arterial hypertension.