Introduction
Methods
Literature search strategy
Selection of papers
Assessment of methodological quality
Data extraction and analysis
Results
Search results
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) |
Methodological aspects
Prognostic value of baseline echocardiographic findings
Serial echocardiographic evaluation
Risk of bias assessment
No. of studies | HR | 95 % CI |
p-value | I2, % | Cochran’s Q (p-value) | |
---|---|---|---|---|---|---|
Presence of pericardial effusion
|
16
|
1.70
|
1.44–1.99
|
< 0.001
|
42
|
25.7 (0.041)
|
> 70 % NYHA class III–IV | 8 | 1.56 | 1.31–1.86 | < 0.001 | 23 | 9.0 (0.249) |
100 % PAH | 12 | 1.62 | 1.34–1.96 | < 0.001 | 45 | 20.1 (0.045) |
Exclusion of CHD | 12 | 1.81 | 1.45–2.25 | < 0.001 | 49 | 21.5 (0.028) |
> 50 % on PAH medication/NR | 10 | 1.86 | 1.40–2.47 | < 0.001 | 60 | 22.3 (0.008) |
Mortality/transplant as outcome | 15 | 1.64 | 1.39–1.94 | < 0.001 | 38 | 22.5 (0.069) |
Right atrial area, per 5 cm
2
increase
|
5
|
1.71
|
1.38–2.13
|
< 0.001
|
55
|
8.9 (0.063)
|
> 70 % NYHA class III–IV | 4 | 1.69 | 1.29–2.21 | < 0.001 | 61 | 7.8 (0.051) |
100 % PAH | 4 | 1.56 | 1.33–1.84 | < 0.001 | 17 | 3.6 (0.306) |
Exclusion of CHD | 4 | 1.69 | 1.29–2.21 | < 0.001 | 61 | 7.8 (0.051) |
> 50 % on PAH medication/NR | 3 | 1.77 | 1.17–2.68 | 0.007 | 74 | 7.7 (0.021) |
Mortality/transplant as outcome | 5 | 1.71 | 1.38–2.13 | < 0.001 | 55 | 8.9 (0.063) |
RV pressure, per 10 mmHg increase
|
7
|
1.18
|
1.01–1.38
|
0.043
|
76
|
25.1 (< 0.001)
|
> 70 % NYHA class III–IV | 4 | 1.33 | 1.00–1.77 | NS | 72 | 10.9 (0.012) |
100 % PAH | 5 | 1.20 | 0.95–1.52 | NS | 75 | 15.8 (0.003) |
Exclusion of CHD | 5 | 1.26 | 1.00–1.59 | NS | 81 | 20.6 (< 0.001) |
> 50 % on PAH medication/NR | 5 | 1.19 | 1.02–1.38 | 0.024 | 66 | 11.8 (0.019) |
Mortality/transplant as outcome | 5 | 1.20 | 0.95–1.52 | NS | 75 | 15.8 (0.003) |
Right atrial pressure, > 15 mmHg
|
6
|
2.45
|
1.56–3.85
|
< 0.001
|
76
|
20.7 (< 0.001)
|
> 70 % NYHA class III–IV | 2 | 1.38 | 0.65–2.92 | NS | 82 | 5.7 (0.017) |
100 % PAH | 6 | 2.45 | 1.56–3.85 | < 0.001 | 76 | 20.7 (< 0.001) |
Exclusion of CHD | 4 | 2.41 | 1.16–4.98 | 0.018 | 82 | 17.0 (< 0.001) |
> 50 % on PAH medication/NR | 0 | – | – | – | – | – |
Mortality/transplant as outcome | 5 | 2.28 | 1.33–3.92 | 0.003 | 72 | 14.2 (0.007) |
TAPSE, per 5 mm decrease
|
7
|
1.72
|
1.34–2.20
|
< 0.001
|
77
|
26.1 (< 0.001)
|
> 70 % NYHA class III–IV | 3 | 1.63 | 1.01–2.63 | 0.047 | 69 | 6.5 (0.039) |
100 % PAH | 4 | 1.67 | 1.15–2.44 | 0.007 | 77 | 12.8 (0.005) |
Exclusion of CHD | 5 | 1.58 | 1.22–2.06 | < 0.001 | 79 | 18.7 (< 0.001) |
> 50 % on PAH medication/NR | 4 | 3.24 | 1.92–5.45 | < 0.001 | 0 | 1.2 (0.756) |
Mortality/transplant as outcome | 5 | 1.76 | 1.22–2.52 | 0.002 | 74 | 15.4 (0.004) |
RV FAC, per 5 % decrease
|
5
|
1.23
|
1.04–1.44
|
0.039
|
60
|
10.1 (0.039)
|
> 70 % NYHA class III–IV | 3 | 1.18 | 0.96–1.44 | NS | 60 | 5.0 (0.080) |
100 % PAH | 2 | 1.06 | 1.04–1.09 | < 0.001 | 0 | 0.0 (0.863) |
Exclusion of CHD | 4 | 1.16 | 1.02–1.32 | 0.026 | 41 | 5.0 (0.168) |
> 50 % on PAH medication/NR | 5 | 1.23 | 1.04–1.44 | 0.039 | 60 | 10.1 (0.039) |
Mortality/transplant as outcome | 3 | 1.18 | 0.96–1.44 | NS | 60 | 5.0 (0.080) |
Tei index, per 0.1 unit increase
|
7
|
1.23
|
1.09–1.40
|
0.001
|
73
|
22.3 (0.001)
|
> 70 % NYHA class III–IV | 3 | 1.22 | 0.99–1.51 | NS | 76 | 8.3 (0.016) |
100 % PAH | 6 | 1.25 | 1.05–1.48 | 0.012 | 58 | 12.0 (0.035) |
Exclusion of CHD | 5 | 1.46 | 1.24–1.72 | < 0.001 | 0 | 2.3 (0.677) |
> 50 % on PAH medication/NR | 2 | 1.25 | 1.16–1.35 | < 0.001 | 0 | 0.5 (0.775) |
Mortality/transplant as outcome | 6 | 1.25 | 1.05–1.48 | 0.012 | 58 | 12.0 (0.035) |