Table. 2—

Long-term survival in pulmonary arterial hypertension (PAH)

	Drug	Patients n	Type	CI L·min⁻¹·m⁻²	NYHA	6MWD m	OA %	Survival %			Comb. %
								1 yr	2 yrs	3 yrs
d'Alonzo [1]	Conservative treatment	194	IPAH				20	68		48
Jing [11]	Conservative treatment	72	IPAH		2.76±0.7		50	68	57	39
Christie [12]	Lung transplant	970	IPAH					67	61	57
Barst [7]	Epoprostenol	18	IPAH	1.90±0.60	3.17±0.5	264±160	100	87	72	63
Sitbon [8]	Epoprostenol	178	IPAH	1.96±0.56	3.33±0.5	240±146		85	70	63
McLaughlin [9]	Epoprostenol	162	IPAH	1.82±0.57	3.54±0.5			88	76	63
Barst [13]	Treprostinil	860	PAH		2.94±0.5			87	78	71	15
Lang [14]	Treprostinil	122	PAH/CTEPH	2.13±0.66	3.20±0.04	305±11	95	89		71	18
Opitz [15]	Inhaled iloprost	76	IPAH	1.80±0.81	2.86±0.6		47	79	70	59	54
Hoeper [16]	i.v. iloprost	79	PAH	1.70±0.60	3.23±0.4	287±112		86	73	59
McLaughlin [17]	Bosentan	169	IPAH	2.35±0.80	2.99±0.5	345±87		96	89	86	30
Hoeper [18]	Bosentan	123	PAH	2.10±0.60	3.20±0.4	308±133	88	93	83	80	43
Provencher [19]	Bosentan	103	IPAH	2.39±0.57	3.12±0.3	319±105	99	92	89	79	44
Sandoval [20]	Atrial septostomy	15	IPAH	2.22±0.46	3.57±0.6	107±127	100	87	87	87
Rich [21]	Calcium channel blockers	17	IPAH (R)	2.60±0.70	2.39±0.5		74	94	94	94
Sitbon [22]	Calcium channel blockers	38	IPAH (LTR)		2.34±1.0	380±112		97	97	97

Data are presented as mean±sd, unless otherwise stated. Summary of large cohorts treated with epoprostenol, treprostinil, iloprost and bosentan in comparison with conservative treatment, lung transplantation, atrial septostomy and calcium channel blockers. CI: cardiac index; NYHA: New York Heart Association; 6MWD: 6-min walk distance; OA: proportion of patients treated with oral anticoagulants; Comb.: proportion of patients started with combination or switched from therapy during the observation period; IPAH: idiopathic PAH; CTEPH: chronic thromboembolic pulmonary hypertension; R: responders; LTR: long-term responders.