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The changing landscape of pulmonary arterial hypertension and implications for patient care

Marius M. Hoeper, J. Simon R. Gibbs
European Respiratory Review 2014 23: 450-457; DOI: 10.1183/09059180.00007814
Marius M. Hoeper
1Dept of Respiratory Medicine, Hannover Medical School and German Centre of Lung Research (DZL), Hannover, Germany. 2National Heart and Lung Institute, Imperial College London, London, UK.
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  • For correspondence: hoeper.marius@mh-hannover.de
J. Simon R. Gibbs
1Dept of Respiratory Medicine, Hannover Medical School and German Centre of Lung Research (DZL), Hannover, Germany. 2National Heart and Lung Institute, Imperial College London, London, UK.
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    Figure 1.

    Survival rates in older (>65 years) compared with younger (18–65 years) patients with idiopathic pulmonary arterial hypertension. a) Expected (- - - -) and observed (––––) survival rates and b) age-/sex-matched survival rates. Reproduced from [13] with permission from the publisher.

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  • Table 1. Demographics of idiopathic pulmonary arterial hypertension patients over time
    Registry[Ref.]Time periodAge yearsFemale1-year survival
    US NIH registry[17, 20]1981–198836±156368
    French registry#[4, 19, 23]2002–200352±156289
    US REVEAL[3, 9, 12]2006–200950±158088
    PH registry of the UK and Ireland#,¶[6]2001–200950±177093
    COMPERA+[13]2007–201165±156092
    New Chinese registry[26]2008–201138±137092
    Czech registry[7]2000–200752±176589¶
    • Data are presented as mean±sd or %, unless otherwise stated. NIH: National Institutes of Health; REVEAL: Registry to Evaluate Early and Long-term PAH Disease Management; PH: pulmonary hypertension; COMPERA: Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension. #: included idiopathic pulmonary arterial hypertension (PAH), familial/heritable PAH and anorexigen-associated PAH; ¶: incident population; +: countries involved included Austria, Belgium, Germany, Italy, the Netherlands, Switzerland and the UK.

  • Table 2. Relationship between reduced diffusing capacity of the lung for carbon monoxide (DLCO) and patient/disease characteristics
    Trip [41]Chandra [44]
    DLCO <45%DLCO ≥45%p-valueDLCO <43%DLCO 43–64%DLCO >64%p-value
    Subjects n48118135135138
    Age years67#46#<0.00155¶47¶45¶<0.0001
    Males50190.012221250.74
    Former smoker58300.07
    Current smoker19180.77
    6MWD m275+390+0.03
    Metabolic equivalents2.63.74.2<0.0001
    NYHA WHO FC I/II21350.05§92526<0.0001ƒ
    NYHA WHO FC III/IV7965917574
    Anti-nuclear antibodies38200.06795532<0.0001
    RAP mmHg7.3+8.1+ns1111110.83
    mPAP mmHg47+52+ns4954540.02
    CI mL·min−1·m−22.3+2.5+ns2220.78
    PVR Wood Unitsns1113130.03
    TPVR dyn·s·cm−51000+1004+
    HR (95% CI)3.8 (2.2–6.6)##<0.0012.7 (1.9–3.9)##<0.001
    2.4 (1.1–5.0)¶¶0.025
    • Data are presented as %, unless otherwise stated. 6MWD: 6-min walking distance; NYHA: New York Heart Association; WHO: World Health Organization; FC: functional class; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; CI: cardiac index; PVR: pulmonary vascular resistance; TPVR: total PVR; HR: hazard ratio; ns: nonsignificant. #: median age at diagnosis; ¶: mean age at study entry; +: approximate value based on data from [42]; §: FC I/II versus III/IV; ƒ: across all groups; ##: univariate analysis; ¶¶: multivariate analysis.

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    • J.S.R. Gibbs
    • M.M. Hoeper
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The changing landscape of pulmonary arterial hypertension and implications for patient care
Marius M. Hoeper, J. Simon R. Gibbs
European Respiratory Review Dec 2014, 23 (134) 450-457; DOI: 10.1183/09059180.00007814

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The changing landscape of pulmonary arterial hypertension and implications for patient care
Marius M. Hoeper, J. Simon R. Gibbs
European Respiratory Review Dec 2014, 23 (134) 450-457; DOI: 10.1183/09059180.00007814
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  • Article
    • Abstract
    • Introduction
    • Changing demographics in PAH patients
    • Clinical characteristics and survival in elderly patients with PAH
    • Implications for management and treatment of PAH in elderly patients
    • Conclusion
    • Acknowledgments
    • Footnotes
    • References
  • Figures & Data
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Subjects

  • Pulmonary pharmacology and therapeutics
  • Pulmonary vascular disease
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