Tab. 1.
Study characteristics
| Study, ref no | Size, n | Age, years | Gender, % female | NYHA class III–IV, % | IPAH/hereditary | Drug/toxin | PAH-CTD | Po-PAH | PAH-CHD | WHO I PAH (other/not specified) | WHO III (lung disease) | WHO IV (CTEPH) | Follow-up duration, months | Events, n (%) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| [11] | 26 | 41 [16–70] | 69 | NR | 100 | - | – | – | – | – | – | – | 24 ± 14 | 16 (62) |
| [12] | 26 | 43 ± 17 | 73 | 58 | 100 | – | – | – | – | – | – | – | NR | 6 (23) |
| [13] | 53 | 45 ± 14 | 72 | 70 | 100 | – | – | – | – | – | – | – | 35 [NR] | 32 (60) |
| [14] | 43 | 37 [14–67] | 70 | 86 | 100 | – | – | – | – | – | – | – | 21 ± 16 | 12 (28) |
| [15] | 25 | 38 ± 13 | 76 | 100 | 100 | – | – | – | – | – | – | – | 12 [0–84] | 13 (52) |
| [16] | 81 | 40 ± 15 | 73 | 100 | 100 | – | – | – | – | – | – | – | 36 ± 15 | 41 (51) |
| [17] | 63 | 55 ± 15 | 83 | 70 | 37 | – | 38 | – | – | – | 21 | 5 | 19 [10–22]a | 23 (37) |
| [18] | 54 | 52 ± 11 | 76 | 76 | 100 | – | – | – | – | – | – | – | 50 [NR] | 12 (22) |
| [19] | 50 | 46 ± 13 | 78 | 42 | 46 | – | 22 | – | – | 4 | – | 28 | 14 [12–18] | 19 (38) |
| [6] | 2716 | 50 ± 17 | 79 | 54 | 49 | 5 | 24 | 5 | 12 | 5 | – | – | 17 [0–24] | 340 (13) |
| [20] | 76 | 61 ± 11 | 84 | 53 | – | – | 100 | – | – | – | – | – | 36 [NR–113] | 42 (55) |
| [21] | 32 | 53 ± 16 | 66 | 91 | 69 | 16 | 6 | – | 9 | – | – | – | 21 [NR] | 17 (53) |
| [22] | 59 | 46 ± 16 | 63 | 66 | 100 | – | – | – | – | – | – | – | 52 [28–79]a | 23 (39) |
| [23] | 72 | 52 ± 16 | 72 | 76 | 100 | – | – | – | – | – | – | – | 38 [14–71]a | 22 (31) |
| [24] | 484 | 52 ± 15 | 75 | 71 | 56 | – | 24 | 11 | 9 | – | – | – | 38 [16–60] | 264 (55) |
| [25] | 50 | 61 ± 11 | 98 | 70 | – | – | 100 | – | – | – | – | – | 16 [9–39] | 25 (50) |
| [26] | 80 | 56 ± 14 | 76 | 72 | 43 | – | 41 | 10 | – | 6 | – | – | 24 [NR] | 33 (41) |
| [27] | 95 | 31 ± 10 | 64 | 56 | 100 | – | – | – | – | – | – | – | 21 ± 15 | 27 (28) |
| [28] | 57 | 52 ± 14 | 28 | 100 | 63 | – | 18 | 11 | – | 5 | – | 3 | 25 ± 29 | 29 (51) |
| [29] | 181 | 39 ± 13 | 67 | 67 | – | – | – | – | 100 | – | – | – | 16 [7–46] | 19 (10) |
| [30] | 154 | 54 ± 9 | 84 | NR | 46 | 1 | 40 | 5 | 6 | 3 | – | – | 36 [17–71]a | 71 (46) |
| [31] | 61 | 48 ± 18 | 84 | 69 | 100 | – | – | – | – | – | – | – | NR | NR |
| [32] | 142 | 59 ± 15 | 65 | 44 | 31 | 9 | 19 | 4 | 9 | 1 | – | 27 | 11 [6–39] | 28 (20) |
| [33] | 577 | 53 ± 15 | 75 | 70 | – | – | – | – | – | 100 | – | – | 57 ± 50 | NR |
| [34] | 406 | 59 ± 16 | 65 | 46 | – | – | – | – | – | 74 | 14 | 12 | 16 [8–20]a | 73 (18) |
| [35] | 32 | 39 ± 15 | 69 | 59 | 22 | – | 16 | – | 53 | – | – | 9 | 14 [8–21] | 15 (47) |
| [36] | 124 | 54 ± 16 | 70 | 92 | – | – | – | – | – | 84 | – | 16 | 36 ± 22 | 31 (25) |
| [37] | 71 | 57 ± 14 | 76 | 75 | 46 | – | 41 | 6 | – | 7 | – | – | 24 [NR] | 20 (28) |
| [38] | 50 | 56 ± 12 | 84 | 72 | 42 | – | 38 | 14 | – | 6 | – | – | 48 [NR] | NR |
| [39] | 102 | 54 ± 16 | 84 | NR | 47 | – | 24 | – | – | 29 | – | – | 44 [22–79]a | 43 (42) |
| [40] | 37 | 46 ± 14 | 76 | 35 | 65 | – | 5 | – | 24 | 5 | – | – | 16 [13–18]a | 7 (19) |
| [41] | 48 | 44 ± 14 | 83 | 100 | 67 | – | 21 | 6 | 6 | – | – | – | 53 [21–80]a | 18 (38) |
| [42] | 91 | 42 ± 14 | 60 | 73 | – | – | – | – | 100 | – | – | – | 46 [4–64] | 24 (26) |
| [43] | 79 | 48 [24–65] | 66 | 92 | 92 | – | 8 | – | – | – | – | – | NR [12–92] | 27 (34) |
| [44] | 121 | 60 ± 14 | 66 | 63 | 39 | – | 36 | 18 | – | 6 | – | – | 37 ± 36 | 49 (40) |
| [45] | 200 | 54 ± 15 | 71 | 50 | 47 | 1 | 33 | 7 | 12 | – | – | – | 43 ± 31 | 106 (53) |
| [46] | 51 | 60 ± 15 | 73 | 71 | 33 | – | 55 | 6 | 6 | – | – | – | 36 ± 24 | 8 (16) |
CHD congenital heart disease, CTD connective tissue disease, CTEPH chronic thromboembolic pulmonary hypertension, HF heart failure, IPAH idiopathic pulmonary arterial hypertension, NYHA New York Heart Association, PAH pulmonary arterial hypertension, PH pulmonary hypertension, SD standard deviation, NR not reported, WHO World Health Organization. ainterquartile range, otherwise reported as median [range] or mean ± SD