Effect of PAH Specific Therapy on Pulmonary Hemodynamics and Six-Minute Walk Distance in Portopulmonary Hypertension: A Systematic Review and Meta-Analysis
Table 2
Regimens of pulmonary arterial hypertension specific therapy used in the included studies [10–21].
1st author and year
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 ()
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.
