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Research ArticleArticle

Echocardiography as an Outcome Measure in Scleroderma-related Pulmonary Arterial Hypertension: A Systematic Literature Analysis by the EPOSS Group

OTYLIA KOWAL-BIELECKA, JEROME AVOUAC, DAVID PITTROW, DOERTE HUSCHER, FRANK BEHRENS, CHRISTOPHER P. DENTON, IVAN FOELDVARI, MARC HUMBERT, MARCO MATUCCI-CERINIC, PETER NASH, CHRISTIAN F. OPITZ, LEWIS J. RUBIN, JAMES R. SEIBOLD, VIBEKE STRAND, DANIEL E. FURST and OLIVER DISTLER
The Journal of Rheumatology January 2010, 37 (1) 105-115; DOI: https://doi.org/10.3899/jrheum.090661
OTYLIA KOWAL-BIELECKA
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JEROME AVOUAC
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DAVID PITTROW
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DOERTE HUSCHER
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FRANK BEHRENS
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CHRISTOPHER P. DENTON
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IVAN FOELDVARI
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MARC HUMBERT
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MARCO MATUCCI-CERINIC
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PETER NASH
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CHRISTIAN F. OPITZ
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LEWIS J. RUBIN
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JAMES R. SEIBOLD
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VIBEKE STRAND
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DANIEL E. FURST
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OLIVER DISTLER
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  • For correspondence: Oliver.Distler@usz.ch
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    Figure 1.

    Results of the systematic literature search.

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    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 PAHPulmonary Fibrosis/Left-heart Disease ExcludedPulmonary Fibrosis/Left-heart Disease Not Excluded
    Right-heart catheterization (RHC)
      mPAP > 25 mm Hg at rest or/andA1A2
      mPAP > 30 mm Hg at exercise
    Doppler echo
      PASP/TG ≥ 45 mm Hg
    RHC
      PASP > 30 mm Hg (in older studies)B1B2
    Doppler echo
      35 mm Hg ≤ PASP/TG < 45 mm HgC1C2
    Other (or not defined)D1D2
    • mPAP: mean pulmonary artery pressure; PASP: pulmonary artery systolic pressure; TG: tricuspid gradient.

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    Table 2.

    Definitions of the OMERACT filter criteria.

    OMERACT Filter CriterionDefinition
    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 validityAbility 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 timeBased 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 treatmentBased 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
    FeasibilityThe 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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    Table 3.

    Studies included into analysis according to the definition of PAH/PH.

    Definition of PAH/PHNo. of Studies Included into Analysis/Analyzed (% of studies included)References
    A15 (14)3, 4, 13†**, 14, 19
    A26 (17)1, 13, 17, 34, 40*, 41
    B10
    B23 (8.6)18, 21, 22
    C10
    C214 (40)2, 12, 15, 23–27, 29, 33, 35, 37–39
    D10
    D29 (26)16, 20, 28, 30–32, 35**, 36, 42
    Total35
    • ↵* Defined by PASP ≥ 35 mm Hg or mean PAP > 20 mm Hg at RHC.

    • ↵** Studies that were duplicated because they contain 2 different groups of PAH/PH patients.

    • ↵† Only DLCO versus PASP subanalysis. Total percentage was higher than 100%, since 2 studies included 2 different groups of PAH/PH.

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    Table 4.

    Validation of echo in PAH-SSc according to the OMERACT filter.

    OMERACT Filter CriterionValidationHighest Quality of PAH Definition
    Truth
      Face validityVNA
      Content validityNVA1
      Criterion validityPVA2
      Construct validityPVA1*/A2
    Discrimination
      Sensitivity to change over timeNDNo studies
      Discrimination capacity over treatmentNDNo studies
      Reliability (reproducibility)PVB2
    FeasibilityUA2
    • ↵* 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.

    StudyNo. (%) of Patients in Whom PASP Could not be EvaluatedReasons for Inability to Evaluate PASPQuality of PAH/PH Definition/involvement of Patients without PAH/PH
    Kiatchoosakun 200718/155 (12)Poor tricuspid velocityC2, patients without PAH/PH included
    Hachulla 2005114/570 (20)Insufficient quality in 23, lack of TR in 91A1 (echo for screen only), patients without PAH/PH included
    Wigley 2005127/669 (19)Tricuspid regurgitant flow could not be identified on DopplerC2, patients without PAH/PH included
    Gindzienska-Sieskiewicz 200527/53 (51)Lack of adequate velocity profiles of tricuspid regurgitationC2, consecutive SSc patients with and without PAH/PH
    Ulanet 200317/80 (21)No detailed dataC2, patients without PAH/PH included
    Giunta 20007/77 (9)—D2, patients without PAH/PH included
    Denton 199713/33 (39)Lack of TRA2, patients without PAH/PH included
    including 6/21 (29)A2, only patients with PAH/PH by RHC
    Murata 199755/135 (43)Lack of adequate velocity profiles of tricuspid regurgitationC2/D2, patients without PAH/PH included
    Battle 19961/34 (3)—C2, patients without PAH/PH included
    Koh 199610/17 (59)No detailed dataC2, only patients with PH by echo (n = 17) confirmed by RHC (n = 4)
    Candell-Riera 199653/72 (74)Insufficient quality of echo images in 9 and lack of TR in 44C2, patients without PAH/PH included; only SlSc
    Murata 199243/71 (61)Insufficient quality of images due to PF or trunkal skin thickening in 6, lack of TR in 30A2, patients without PAH/PH included
    Smith 197942/54 (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.

    • View popup
    Table 6.

    Studies required for further validation of echo as an outcome measure in PAH-SSc.

    OMERACT Filter CriterionValidationType of Study
    Truth
      Face validityVNone
      Content validityNVNone
      Criterion validityPVCross-sectional echo vs RHC, e.g., baseline from RCT
      Construct validityPVCross-sectional echo vs other outcomes, e.g., baseline from RCT
    Discrimination
      Sensitivity to change over timeNDLongitudinal echo vs RHC, e.g., placebo group of RCT
      Discrimination capacity over treatmentNDLongitudinal echo vs RHC, e.g., verum group of RCT
      Reliability (reproducibility)PVRepetition of echo within a short time by several in vestigators (inter-and intra observer variability)
    FeasibilityUCross-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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Echocardiography as an Outcome Measure in Scleroderma-related Pulmonary Arterial Hypertension: A Systematic Literature Analysis by the EPOSS Group
OTYLIA KOWAL-BIELECKA, JEROME AVOUAC, DAVID PITTROW, DOERTE HUSCHER, FRANK BEHRENS, CHRISTOPHER P. DENTON, IVAN FOELDVARI, MARC HUMBERT, MARCO MATUCCI-CERINIC, PETER NASH, CHRISTIAN F. OPITZ, LEWIS J. RUBIN, JAMES R. SEIBOLD, VIBEKE STRAND, DANIEL E. FURST, OLIVER DISTLER
The Journal of Rheumatology Jan 2010, 37 (1) 105-115; DOI: 10.3899/jrheum.090661

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Echocardiography as an Outcome Measure in Scleroderma-related Pulmonary Arterial Hypertension: A Systematic Literature Analysis by the EPOSS Group
OTYLIA KOWAL-BIELECKA, JEROME AVOUAC, DAVID PITTROW, DOERTE HUSCHER, FRANK BEHRENS, CHRISTOPHER P. DENTON, IVAN FOELDVARI, MARC HUMBERT, MARCO MATUCCI-CERINIC, PETER NASH, CHRISTIAN F. OPITZ, LEWIS J. RUBIN, JAMES R. SEIBOLD, VIBEKE STRAND, DANIEL E. FURST, OLIVER DISTLER
The Journal of Rheumatology Jan 2010, 37 (1) 105-115; DOI: 10.3899/jrheum.090661
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