Table 1.
Baseline Characteristics of Patients in Included Studies.
| Study (Design) |
Number of Patients (Single or Multiple Centers) |
Male Patients n (%) |
Mean Age (Years) |
Person Years Follow-Up | WHO FC I n (%) |
WHO FC II n (%) |
WHO FC III n (%) |
WHO FC IV n (%) |
Mean Number of Catheterization Procedures | Proportion on Vasodilator Therapy |
Mean Baseline 6MWD (m) |
Mean Baseline mPAP (mmHg) |
Mean Baseline CI (L/min/m2) |
Mean Baseline PVR (Wood units) |
|||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Balloon Pulmonary Angioplasty | |||||||||||||||||||||||||||
|
Feinstein et al., 2001 (Observational) |
18 (Single) |
NR | 52 | 51.3 | NR | NR | NR | NR | 2.7 | NR | 191.1 | 42.0 | 2.0 | 22.0 | |||||||||||||
|
Roik et al., 2016 (Observational) |
10 (Single) |
4 (40%) |
81 | 15.4 | 0 | 0 |
7 (70%) |
3 (30%) |
3.9 |
6 (60%) |
210.0 | 41.5 | 2.3 | 8.9 | |||||||||||||
|
Moriyama et al., 2017 (Observational) |
53 (Single) |
13 (24.5%) |
63 | NR | 0 |
11 (21%) |
36 (68%) |
6 (11%) |
6.0 | NR | 351.4 | 37.2 | 2.2 | 8.4 | |||||||||||||
|
Ogawa et al., 2017 (Observational) |
308 (Multiple) |
62 (20.1%) |
62 | 364.0 | 0 |
56* (18%) |
192* (62%) |
43* (14%) |
8.3 |
222 (71%) |
318.1 | 43.2 | 2.6 | 10.7 | |||||||||||||
|
Yamasaki et al., 2017 (Observational) |
20 (Single) |
4 (20.0%) |
62 | NR | 0 |
2 (10%) |
17 (85%) |
1 (5%) |
2.7 |
20 (100%) |
391.0 | 42.6 | 3.1 | 8.0 | |||||||||||||
|
Kriechbaum et al., 2018 (Observational) |
51 (Single) |
23 (45.1%) |
63 | 25.5 | 0 |
2 (4%) |
31 (61%) |
18 (35%) |
5.2 |
29 (57%) |
367.2 | 39.5 | NR | 6.5 | |||||||||||||
|
Kurzyna et al., 2018 (Observational) |
31 (Single) |
NR | NR | 32.3 | 0 |
1 (3%) |
23 (74%) |
7 (23%) |
NR | NR | 306.0 | 50.7 | 2.3 | 10.3 | |||||||||||||
|
Kwon et al., 2018 (Observational) |
15 (Single) |
8 (53.3%) |
53 | 11.3 | 0 |
6 (40%) |
5 (33%) |
4 (27%) |
3.5 |
9 (60%) |
387.0 | NR | 2.9 | 7.6 | |||||||||||||
|
Velazquez et al., 2018 (Observational) |
46 (Single) |
32 (69.6) |
14 (30%) |
54.8 | 0 |
5/43 (12%) |
30/43 (70%) |
8/43 (19%) |
NR |
46 (100%) |
394.5 | 49.5 | 2.3 | 10.1 | |||||||||||||
| Balloon Pulmonary Angioplasty (continued) | |||||||||||||||||||||||||||
|
Yamagata et al., 2018 (Observational) |
19 (Single) |
3 (15.8%) |
68 | NR | 0 |
3 (16%) |
16 (84%) |
0 | 3.2 | NR | 308.1 | 40.1 | NR | 7.5 | |||||||||||||
|
Brenot et al., 2019 (Observational) |
184 Multiple) |
94 (51.1%) |
63 | 93.5 |
2 (1%) |
64 (35%) |
109 (59%) |
9 (5%) |
5.4 | 57% | 396 | 44.1 | 2.7 | 7.6 | |||||||||||||
| Pulmonary Vasodilators | |||||||||||||||||||||||||||
|
Ghofrani et al., 2003 (Observational) |
12 (Single) |
7 (58%) |
NR | 6.5 | NR | NR | NR | NR |
Sildenafil PO (50 mg TID/ 6 months) |
312 | NR | 2.0 | NR | ||||||||||||||
|
Scelsi et al., 2004 (Observational) |
11 (Single) |
6 (55%) |
50 | 11.4 | 0 | 0 |
11 (100%) |
0 |
Epoprostenol IV (12.7 + 6.8 ng/kg/min/ 12 months) |
253 | 44.0 | NR | 12.0 | ||||||||||||||
|
Bonderman et al., 2005 (Observational) |
16 (Single) |
7 (44%) |
70 | 8.0 | 0 |
10 (63%) |
4 (25%) |
2 (13%) |
Bosentan PO (125 mg BID/ 6 months) |
299 | 56.0 | 1.9 | 8.9 | ||||||||||||||
|
Hoeper et al., 2005 (Observational) |
18 (Multiple) |
11 (61%) |
60 | 4.5 | 0 |
2 (11%) |
14 (88%) |
2 (11%) |
Bosentan PO (125 mg BID/ 3 months) |
340 | 47.0 | 2.1 | 11.4 | ||||||||||||||
|
Hughes et al., 2006 (Observational) |
47 (Multiple) |
20 (43%) |
56 | 53.3 | 0 |
10 (21%) |
32 (68%) |
5 (11%) |
Bosentan PO (62.5 mg BID/ 12 months) |
291 | NR | NR | NR | ||||||||||||||
|
Vizza et al., 2006 (Observational) |
8 (Single) |
1 (13%) |
44 | 4.0 | 0 |
3 (38%) |
4 (50%) |
1 (13%) |
Beraprost PO (275 + 47 μg/ 6 months) |
313 | 48.0 | 2.4 | 11.0 | ||||||||||||||
|
Cabrol et al., 2007 (Observational) |
27 (Single) |
13 (48%) |
51 | 27.8 | 0 | 0 |
20 (74%) |
7 (26%) |
Epoprostenol IV (16 + 2.8 ng/kg/min/ 20 months) |
265 | 52.0 | 2.1 | NR | ||||||||||||||
| Pulmonary Vasodilators (continued) | |||||||||||||||||||||||||||
|
Reichenberger et al., 2007 (Observational) |
104 (Single) |
45 (43%) |
62 | 104.0 | 0 |
8 (8%) |
76 (73%) |
20 (19%) |
Sildenafil PO (50 mg TID/ 12 months) |
310 | 44.2 | NR | 10.8 | ||||||||||||||
|
Segovia Cubero et al., 2007 (Observational) |
6 (Single) |
1 (17%) |
60 | 7.5 | NR | NR | NR | NR |
Bosentan PO (125 mg BID/ 15 months) |
230 | 55.0 | 1.9 | 12.6 | ||||||||||||||
|
Seyfarth et al., 2007 (Observational) |
12 (Single) |
5 (42%) |
57 | 24.0 | 0 | 0 |
12 (100%) |
0 |
Bosentan PO (125 mg BID/ 24 months) |
319 | 45.8 | 2.2 | 12.6 | ||||||||||||||
|
Skoro-Sajer et al., 2007 (Observational) |
25 (Single) |
9 (36%) |
60 | 50.0 | 0 | 0 |
11 (44%) |
14 (56%) |
Treprostinil IV (28 + 10 ng/kg/min 19 months) |
260 | 41.0 | NR | 11.6 | ||||||||||||||
|
Jais et al., 2008 (RCT) |
77 (Multiple) |
22 (29%) |
63 | 25.7 | 0 |
22 (29%) |
51 (66%) |
3 (4%) |
Bosentan PO (125 mg BID/ 4 months) |
340 | NR | NR | 9.7 | ||||||||||||||
|
Rossi et al., 2008 (Observational) |
9 (Single) |
2 (22%) |
67 | 4.5 | 0 | 0 |
8 (89%) |
1 (11%) |
Sildenafil PO (100 mg TID/ 6 months) |
244 | NR | 2.30 | 14.4 | ||||||||||||||
|
Suntharalingam et al., 2008 (RCT) |
9 (Single) |
2 (22%) |
49 | 2.3 | 0 |
3 (33%) |
6 (67%) |
0 |
Sildenafil PO (40 mg TID/ 3 months) |
331 | 45.0 | NR | 10.1 | ||||||||||||||
|
Post et al., 2009 (Observational) |
18 (Single) |
11 (61%) |
63 | 49.5 | 0 |
15 (83%) |
3 (17%) |
0 |
Bosentan PO (125 mg BID/ 31 months) |
405 | 49.9 | 2.20 | 7.8 | ||||||||||||||
|
Vassallo et al., 2009 (Observational) |
17 (Multiple) |
2 (12%) |
65 | 17.0 | 0 |
1 (6%) |
14 (82%) |
2 (12%) |
Bosentan PO (125 mg BID/ 12 months) |
297 | 49.9 | 2.30 | NR | ||||||||||||||
|
Ghofrani et al., 2010 (Observational) |
41 (Multiple) |
23 (56%) |
63 | 10.3 | 0 |
10 (24%) |
31 (76%) |
0 |
Riociguat PO (2.5 mg TID/ 3 months) |
387 | 43.8 | 2.31 | 8.6 | ||||||||||||||
| Pulmonary Vasodilators (continued) | |||||||||||||||||||||||||||
|
Ghofrani et al., 2013 (RCT) |
173 (Multiple) |
55 (32%) |
59 | 57.7 |
3 (2%) |
55 (32%) |
107 (62%) |
8 (5%) |
Riociguat PO (2.5 mg TID/ 4 months) |
342 | 44.0 | NR | 9.9 | ||||||||||||||
|
Ghofrani et al., 2017 (RCT) |
40 (Multiple) |
14 (35%) |
58 | 20.0 | 0 |
12 (30%) |
28 (70%) |
0 |
Macitentan PO (10 mg daily/ 4 months) |
353 | 56.0 | 1.90 | 11.6 | ||||||||||||||
|
Yamamoto et al., 2017 (Observational) |
23 (Single) |
3 (12%) |
66 | 23.0 | 0 |
20 (87%) |
3 (13%) |
0 |
Riociguat (2.5 mg TID/ 12 months) |
373 | 38 | 3.0 | 6.7 | ||||||||||||||
|
Sadushi-Kolici et al., 2018 (RCT) |
105 (Multiple) |
56 (53%) |
64 | 52.5 | 0 |
6 (6%) |
91 (87%) |
8 (8%) |
Treprostinil SC (30 ng/kg/min/ 3 months) |
303 | 47.0 | 2.10 | 10.3 | ||||||||||||||
|
Escribano-Subias et al., 2019 (RCT) |
15 (Multiple) |
NR | NR | 5.0 | NR | NR | NR | NR |
Ambrisentan (5-10 mg daily/ 4 months) |
NR | NR | NR | NR | ||||||||||||||
|
Van Thor et al., 2019 (Observational) |
36 (Multiple) |
18 (50%) |
65 | 545.2 | 0 |
16 (46%) |
18 (51%) |
1 (3%) |
Riociguat (2.5 mg TID/ mean 28 months) |
337 | 38.1 | NR | 6.1 | ||||||||||||||
Legend: *: Incomplete reporting of baseline characteristics, μg: Micrograms, 6MWD: Six-Minute Walk Distance, BID: Bis in Die (Twice Daily), CI: Cardiac Index, IV: Intravenous, L/min/m2: Liters per minute per meters squared, m: meters, mmHg: Millimeters of mercury, mPAP: Mean Pulmonary Artery Pressure, n: number, ng/kg/min: Nanograms/kilograms/minute, NR: Not Reported, PO: Per OS (oral), PVR: Pulmonary Vascular Resistance, RCT: Randomized Controlled Trial, SC: Subcutaneous, TID: Ter in Die (Three Times Daily), WHO FC: World Health Organization Functional Class.