Article Figures & Data
Figures
- Figure 1.
Results of the systematic literature search.
Tables
- Table 1.
Quality assessment of studies according to the definition of pulmonary arterial hypertension (PAH) and the exclusion of other forms of pulmonary hypertension. For detailed definition of quality criteria A-D and category 1/2 see Materials and Methods. If only A1 studies were available, specific OMERACT criteria for echo were considered validated (V) or not valid (NV). Echo was considered partially validated if studies other than A1 indicated that echo was valid.
Definition of PAH Pulmonary Fibrosis/Left-heart Disease Excluded Pulmonary Fibrosis/Left-heart Disease Not Excluded Right-heart catheterization (RHC) mPAP > 25 mm Hg at rest or/and A1 A2 mPAP > 30 mm Hg at exercise Doppler echo PASP/TG ≥ 45 mm Hg RHC PASP > 30 mm Hg (in older studies) B1 B2 Doppler echo 35 mm Hg ≤ PASP/TG < 45 mm Hg C1 C2 Other (or not defined) D1 D2 -
mPAP: mean pulmonary artery pressure; PASP: pulmonary artery systolic pressure; TG: tricuspid gradient.
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OMERACT Filter Criterion Definition Truth Face validity (credibility) Overall appropriateness of method to be used for evaluation of the outcome, as assessed by investigators and clinicians Content validity (comprehensiveness) Ability of the outcome measure to include or predict all those components of health status relevant to the intervention being assessed. Thus, it was evaluated whether echo measurements cover the whole spectrum of PAH-SSc patients and whether its measurements are specific for PAH Criterion validity (accuracy) Ability of the outcome measure to reflect best available estimate of true clinical status of the patient. Thus, criterion validity was assessed through comparisons/correlation of echo with RHC as the “gold standard” technique in PAH/PH Construct validity Ability of the outcome measure to match with the hypothesized expectations of the investigator compared with other indirect assessments. Thus, construct validity was assessed through assessment of convergent and divergent validity based on associations/correlations of echo measures with other clinically relevant disease measures. Since echo has been used frequently as a diagnostic tool in multiple research studies, only associations/correlations with measures defined by PAH experts as important for evaluation of PAH-SSc patients were taken into account for this analysis13 Discrimination Sensitivity to change over time Based on calculation of standardized response mean (SRM) using repeated measures performed in a given population at 2 different timepoints without therapeutic intervention in between Discrimination capacity over treatment Based on calculation of effect size in randomized controlled trials or SRM in open-label trials Reliability (reproducibility) Based on evaluation of intra-and interclass correlations Feasibility The measure’s ease of use, cost-effectiveness, availability in different centers, and overall usefulness -
PAH-SSc: pulmonary arterial hypertension-systemic sclerosis; RHC: right-heart catheterization.
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Definition of PAH/PH No. of Studies Included into Analysis/Analyzed (% of studies included) References A1 5 (14) 3, 4, 13†**, 14, 19 A2 6 (17) 1, 13, 17, 34, 40*, 41 B1 0 B2 3 (8.6) 18, 21, 22 C1 0 C2 14 (40) 2, 12, 15, 23–27, 29, 33, 35, 37–39 D1 0 D2 9 (26) 16, 20, 28, 30–32, 35**, 36, 42 Total 35 OMERACT Filter Criterion Validation Highest Quality of PAH Definition Truth Face validity V NA Content validity NV A1 Criterion validity PV A2 Construct validity PV A1*/A2 Discrimination Sensitivity to change over time ND No studies Discrimination capacity over treatment ND No studies Reliability (reproducibility) PV B2 Feasibility U A2 -
↵* For some aspects of construct validity only (association between pericardial effusion and mortality, tricuspid regurgitant jet velocity, and dyspnea). V: valid: A criterion was judged validated if appropriate information was available from studies including exclusively PAH-SSc patients (quality definition A1, see Table 1). Exception is face validity, which is evaluated by the judgement of experts as an appropriate measure rather than by specific studies. NV: not valid: Similarly, a criterion was judged not valid if appropriate information was available from studies including exclusively PAH-SSc patients (quality definition A1). PV: partially validated: A criterion was judged partially validated if data from studies lower than quality level A1 indicated that the criterion was validated. U: unclear, possibly not valid: A criterion was judged unclear/possibly not valid if data from studies lower than quality level A1 indicated that the criterion was not valid. NA: not applicable; ND: no data.
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- Table 5.
Numbers of SSc patients in whom pulmonary artery systolic pressure (PASP) could not be evaluated by echo-Doppler in studies identified by the literature search.
Study No. (%) of Patients in Whom PASP Could not be Evaluated Reasons for Inability to Evaluate PASP Quality of PAH/PH Definition/involvement of Patients without PAH/PH Kiatchoosakun 2007 18/155 (12) Poor tricuspid velocity C2, patients without PAH/PH included Hachulla 2005 114/570 (20) Insufficient quality in 23, lack of TR in 91 A1 (echo for screen only), patients without PAH/PH included Wigley 2005 127/669 (19) Tricuspid regurgitant flow could not be identified on Doppler C2, patients without PAH/PH included Gindzienska-Sieskiewicz 2005 27/53 (51) Lack of adequate velocity profiles of tricuspid regurgitation C2, consecutive SSc patients with and without PAH/PH Ulanet 2003 17/80 (21) No detailed data C2, patients without PAH/PH included Giunta 2000 7/77 (9) — D2, patients without PAH/PH included Denton 1997 13/33 (39) Lack of TR A2, patients without PAH/PH included including 6/21 (29) A2, only patients with PAH/PH by RHC Murata 1997 55/135 (43) Lack of adequate velocity profiles of tricuspid regurgitation C2/D2, patients without PAH/PH included Battle 1996 1/34 (3) — C2, patients without PAH/PH included Koh 1996 10/17 (59) No detailed data C2, only patients with PH by echo (n = 17) confirmed by RHC (n = 4) Candell-Riera 1996 53/72 (74) Insufficient quality of echo images in 9 and lack of TR in 44 C2, patients without PAH/PH included; only SlSc Murata 1992 43/71 (61) Insufficient quality of images due to PF or trunkal skin thickening in 6, lack of TR in 30 A2, patients without PAH/PH included Smith 1979 42/54 (78) — D2, patients without PAH/PH included -
* Data reported for visualization of pulmonic valve only. Echo: echocardiography; PAH: pulmonary arterial hypertension; PASP: pulmonary artery systolic pressure; PH: pulmonary hypertension; TR: tricuspid regurgitation; RHC: right-heart catheterization; lSSc: limited cutaneous systemic sclerosis.
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- Table 6.
Studies required for further validation of echo as an outcome measure in PAH-SSc.
OMERACT Filter Criterion Validation Type of Study Truth Face validity V None Content validity NV None Criterion validity PV Cross-sectional echo vs RHC, e.g., baseline from RCT Construct validity PV Cross-sectional echo vs other outcomes, e.g., baseline from RCT Discrimination Sensitivity to change over time ND Longitudinal echo vs RHC, e.g., placebo group of RCT Discrimination capacity over treatment ND Longitudinal echo vs RHC, e.g., verum group of RCT Reliability (reproducibility) PV Repetition of echo within a short time by several in vestigators (inter-and intra observer variability) Feasibility U Cross-sectional, e.g., baseline from RCT -
For definition of validation see Table 4. RCT: randomized controlled trial; RHC: right-heart catheterization. V: valid; NV: not valid; PV: partially validated; U: unclear.
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