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Chronic post-embolic pulmonary hypertension: a new target for medical therapies?

Marion Delcroix
European Respiratory Review 2013 22: 258-264; DOI: 10.1183/09059180.00003513
Marion Delcroix
Respiratory Division, University Hospitals and Dept of Clinical and Experimental Medicine, University of Leuven, Leuven, Belgium.
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  • For correspondence: marion.delcroix@uzleuven.be
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    Figure 1.

    Characteristics of chronic post-embolic pulmonary hypertension.

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  • Table 1. Short-term effects of medical treatment in chronic post-embolic pulmonary hypertension
    TreatmentAdministrationFirst author [Ref.]StudyDuration monthsPatients nNYHA-FC6MWD mEffect mPVR dyn·s−1·cm−5Effect %
    Epoprostenoli.v.Cabrol [6]323III–IV280±112+6629±7#,¶-21
    TreprostinilSubcutaneouslySkoro-Sajer [7]6 or 1925III–IV260±111+59924±347-13
    IloprostInhaledOlschewski [8]RCT357III–IVNAnsNAns
    SildenafilBy mouthGhofrani [9]612ns312±30+541935±228+-30
    SildenafilBy mouthReichenberger [10]3104II–IV310±11+51863±38-12
    SildenafilBy mouthSuntharalingam [11]RCT319II–III339±58+18 (ns)734±363-27
    BosentanBy mouthHoeper [12]319II–IV340±102+73914±329-33
    BosentanBy mouthHughes [13]320II–IV262±106+451165±392#-21
    BosentanBy mouthBonderman [14]616II–IV299±131+92712±213NA
    BosentanBy mouthSeyfarth [15]612III319±85+721008±428NA
    BosentanBy mouthJaïs [16]RCT4157II–III342±84+2 (ns)783-24
    RiociguatBy mouthGhofrani [17]OL341II–III390 (330–441)+55691 (533–844)-29
    RiociguatBy mouthGhofrani [18]RCT4261II–III349+46785-31
    • Data are presented as mean±sd or median (interquartile range), unless otherwise stated. NYHA-FC: New York Heart Association functional class; 6MWD: 6-min walking distance; PVR: pulmonary vascular resistance; RCT: randomised controlled trial; OL: open label; NA: not available; ns: not significant. #: total PVR; ¶: in Woods Units·m2; +: in dyn·s−1·cm−5·m−2.

  • Table 2. Long-term survival outcomes in medically treated chronic post-embolic pulmonary hypertension patients
    TreatmentAdministrationFirst author [Ref.]Patients nNYHA-FC6MWD mSurvival %
    Year 1Year 2Year 3Year 5
    BosentanOrallyHughes [22]47II–IV291±1169690NANA
    BosentanOrallySeyfarth [15]12III319±85100100NANA
    Epoprostenoli.v.Cabrol [6]27III–IV265±117735941NA
    TreprostinilSubcutaneouslySkoro-Sajer [7]25III–IV260±11180808053
    Medical#Suntharalingam [23]35NANA77NA53NA
    Medical¶Condliffe [20]148II–IV239±13382NA7555
    Medical+Simonneau [21]275II–IV315887970NA
    • Data are presented as mean±sd, unless otherwise stated. NYHA-FC: New York Heart Association functional class; 6MWD: 6-min walking distance; NA: not available. #: 68% received medical treatment; ¶: 86% received medical treatment; +: 61% received medical treatment.

  • Table 3. Long-term survival rate in operated chronic post-embolic pulmonary hypertension patients
    TreatmentFirst author [Ref.]Patients nNYHA-FC6MWD mSurvival %
    Year 1Year 2Year 3Year 5
    PEAArchibald [24]532NDND88848176
    PEACorsico [25]157II–IVNDNANANA84
    PEACondliffe [20]236II–IV243±13388NA7675
    PEASimonneau [21]404II–IV340939189NA
    • Data are presented as mean±sd, unless otherwise stated. PEA: pulmonary endarterectomy; NYHA-FC: New York Heart Association functional class; 6MWD: 6-min walking distance; ND: not determined; NA: not available.

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Chronic post-embolic pulmonary hypertension: a new target for medical therapies?
Marion Delcroix
European Respiratory Review Sep 2013, 22 (129) 258-264; DOI: 10.1183/09059180.00003513

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Chronic post-embolic pulmonary hypertension: a new target for medical therapies?
Marion Delcroix
European Respiratory Review Sep 2013, 22 (129) 258-264; DOI: 10.1183/09059180.00003513
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  • Article
    • Abstract
    • Introduction
    • Rationale for medical therapy in CTEPH
    • Short-term effects of medical therapy
    • Long-term survival effects of medical therapy
    • Patient selection for medical therapy
    • Conclusions
    • Footnotes
    • References
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  • Pulmonary pharmacology and therapeutics
  • Pulmonary vascular disease
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