Introduction
Methods
Literature search strategy
Selection of papers
Assessment of methodological quality
Data extraction and analysis
Results
Search results
Study population | WHO classification (%) | PAH medication (%) | Outcome | ||||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Author, year (ref) | Size (n) | Age (mean ± SD) | Sex (% female) | NYHA class III-IV (%) | Idiopathic PAH | Hereditary PAH | Drug/toxin | PAH-CTD | PAH-HIV | Po-PAH | PAH-CHD | WHO I PAH (other/not specified) | WHO II (left heart disease) | WHO III (lung disease) | WHO IV (CTEPH) | WHO V (unclear/multifactorial) | Calcium antagonist | PD5-inhibitor | Endothelin receptor antagonist | Prostacyclin analogue | Endpoint | Follow-up duration (months, mean ± SD or median [IQR]) | Events n (%) |
Gan, 2007 [18] | 70 | 50 ± 15 | 79 | nr | 70 | _ | _ | 23 | 3 | _ | _ | 4 | _ | _ | _ | _ | 4 | 6 | 51 | 29 | Death | nr | 18 (26) |
Van Wolferen, 2007 [19] | 64 | 43 ± 13 | 73 | 89 | 100 | _ | _ | _ | _ | _ | _ | _ | _ | _ | _ | _ | 8 | 6 | 39 | 47 | Death, transplant | 32 ± 16 | 19 (30) |
Van de Veerdonk, 2011 [20] | 110 | 53 ± 15 | 76 | 52 | 66 | 6 | 3 | 18 | 2 | 5 | _ | _ | _ | _ | _ | _ | 3 | 15 | 35 | 14 | Death | 12 [10–16] | 13 (12) |
Freed, 2012 [21] | 58 | 53 ± 14 | 74 | nr | 41 | _ | _ | _ | _ | _ | _ | 34 | 14 | 2 | 3 | 5 | [ | 31 | ] | 31 | Death, transplant, admission for HF | 10 ± 6 | 19 (33) |
Yamada, 2012 [22] | 41 | 39 ± 14 | 71 | 51 | 100 | _ | _ | _ | _ | _ | _ | _ | _ | _ | _ | _ | 22 | 88 | 54 | 34 | Death | 45 ± 26 | 4 (10) |
Cho, 2014 [23] | 37 | 46 ± 14 | 76 | 35 | 65 | _ | _ | 5 | _ | _ | 24 | 5 | _ | _ | _ | _ | 8 | 21 | 62 | 14 | Death, admission for HF | 16 [13–18] | 7 (19) |
Swift, 2014 [24] | 80 | 59 ± 17 | 60 | 66 | 100 | _ | _ | _ | _ | _ | _ | _ | _ | _ | _ | _ | nr | nr | nr | nr | Death | 32 ± 14 | 23 (29) |
Swift, 2014 [25] | 79 | 62 ± 16 | 61 | nr | 44 | _ | _ | 47 | _ | _ | 9 | _ | _ | _ | _ | _ | nr | nr | nr | nr | Death | nr | 25 (32) |
Methodological aspects
Selection bias | Information bias: defined and measured appropriately? | Statistical calculation of effect size | |||||||||
---|---|---|---|---|---|---|---|---|---|---|---|
Author, year | Study design | Missing data | Loss to follow-up | Description of CMR protocol | Measurement of CMR findings | Definition and measurement of outcome | Description of statistical analysis | Effect size: hazard ratios | Treatment of continuous predictors | Multivariable adjustment | Multivariable analysis appropriate |
Gan, 2007 [18] | − | nr | + | + | + | + | + | + | + | ± | − |
Van Wolferen, 2007 [19] | + | + | + | ± | ± | + | ± | + | + | ± | − |
Van de Veerdonk, 2011 [20] | + | ± | + | + | + | + | + | + | + | ± | − |
Freed, 2012 [21] | + | ± | + | ± | + | + | + | + | + | ± | − |
Yamada, 2012 [22] | − | + | + | + | ± | + | ± | + | + | − | NA |
Cho, 2014 [23] | − | nr | nr | ± | + | + | ± | + | − | − | NA |
Swift, 2014 [24] | − | + | + | ± | + | ± | + | + | + | − | NA |
Swift, 2014 [25] | − | + | nr | + | + | ± | + | + | + | + | + |
Prognostic value of baseline CMR findings
CMR finding | Total studies (n) | Gan, 2007 [18] | Van Wolferen, 2007 [19] | Van de Veerdonk, 2011 [20] | Freed, 2012 [21] | Yamada, 2012 [22] | Cho, 2014 [23] | Swift, 2014 [24] | Swift , 2014 [25] |
---|---|---|---|---|---|---|---|---|---|
RV ejection fraction | 6 | xa
| xa
| x | x | x | x | ||
RV end-diastolic volume | 5 | xa
| xa
| x | x | x | |||
RV end-systolic volume | 4 | xa
| x | x | x | ||||
RV stroke volume | 3 | x | x | x | |||||
PA flow stroke volume index | 2 | xa
| x | ||||||
PA flow cardiac index | 2 | xa
| x | ||||||
RV mass | 3 | xa
| x | x | |||||
LV ejection fraction | 5 | xa
| xa
| x | x | x | |||
LV end-diastolic volume | 5 | xa
| xa
| x | x | x | |||
LV end-systolic volume | 4 | xa
| x | x | x | ||||
LV stroke volume | 2 | xa
| x | ||||||
LV mass | 3 | xa
| x | x | |||||
RV wall thickness | 1 | xa
| |||||||
LV wall thickness | 1 | xa
| |||||||
Ventricular mass index (RV/LV mass) | 1 | x | |||||||
RA volume | 1 | x | |||||||
LA volume | 1 | x | |||||||
PA relative area change | 1 | x | |||||||
RV insertion points late gadolinium enhancement | 1 | x | |||||||
Full width at half maximum of the bolus passage | 1 | x | |||||||
Pulmonary transit time | 1 | x |