Table 1.

Demographics and clinical characteristics at enrollment.

		IPAH (n = 1481)		PAH-CHD Total (n = 680)		ES Untreated (n = 80)		ES Treated (n = 240)		Non-ES-PAH Treated (n = 167)		Fontan Treated (n = 7)
Age		66.3 ± 15.5		45.5 ± 16.8		40.1 ± 14.6		43.8 ± 15.1		47.6 ± 18.3		31.9 ± 9.4
Sex	male	604	40.8%	227	33.4%	26	32.5%	76	31.7%	55	32.9%	3	42.9%
	female	877	59.2%	453	66.6%	54	67.5%	164	68.3%	112	67.1%	4	57.1%
FC	unknown	78	5.3%	80	11.8%	30	37.5%	19	7.9%	14	8.4%	4	57.1%
	I	3	0.2%	22	3.2%	1	1.3%	8	3.3%	5	3.0%	0	0%
	II	178	12.0%	159	23.4%	11	13.8%	65	27.1%	44	26.3%	3	42.9%
	III	997	67.3%	392	57.6%	36	45.0%	136	56.7%	101	60.5%	0	0%
	IV	225	15.2%	27	4.0%	2	2.5%	12	5.0%	3	1.8%	0	0%
6-min-walk distance (m)		294 ± 123		367 ± 120		392 ± 118		354 ± 121		381 ± 120		475 ± 50

Abbreviations: CHD, congenital heart disease; ES, Eisenmenger syndrome; FC, functional class according to Perloff; IPAH, idiopathic pulmonary arterial hypertension; non-ES-PAH, patients with congenital heart disease-associated pulmonary arterial hypertension but without Eisenmenger syndrome; PAH-CHD; congenital heart disease-associated pulmonary arterial hypertension. Age and 6-min-walk distance are given in mean ± SD.