Table 2.

Regimens of pulmonary arterial hypertension specific therapy used in the included studies [10–21].

1st author and year	N	Baseline mPAP in mm Hg Mean ± SD	PAH specific therapy	Dose Median (range) unless otherwise specified	Duration of therapy in months Total or median (range)
Krowka 1999	7	50 ± 13.4	Epoprostenol	11 (7–48) ng/kg/min	6 (3–30)
Hoeper 2005	11	53 ± 9	Bosentan	250 mg/day	12
Reichenberger 2006	12	55 ± 11	Sildenafil  ±iloprost	150 mg/day 30 mcg/day (n = 5)	12
Sussman 2006	8	43	Epoprostenol	2–8 ng/kg/min	4.5 (2–15)
Fix 2007	14	47.9 ± 8.5	Epoprostenol ± another*	29 (6.5–50.5) ng/kg/min	15.4 (6.2–69.8)
Hoeper 2007	13	53 ± 8	Bosentan	250 mg/day	12
Hoeper 2007	11	50 ± 10	Iloprost	30 mcg/day	12
Gough and White 2009	9	47.6 ± 9.9	Sildenafil	150 (60–400) mg/day	5.6 (3.2–9.4)
Hemnes 2009	10	47.8 ± 12.1	Sildenafil	60–150 mg/day	12
Melgosa 2010	12	55 ± 10	Iloprost	30 mcg/day	12
Halank 2011	5	47 ± 6	Ambrisentan	5 or 10 mg/day	12
Hollatz 2012	11	44.4 ± 5.5	Sildenafil  +/or SQ trepostinil	120 (60–150) mg/day 32 (19–53) ng/kg/min	7 (1–48)
Savale 2013	34	50 ± 10	Bosentan	250 mg/day	4–12

^*Sildenafil (and occasionally bosentan, inhaled iloprost, or subcutaneous treprostinol) was added if the response to epoprostenol was considered by the treating physician to be inadequate, or if side effects greatly limited the ability to achieve an adequate infusion rate of epoprostenol.