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The use of combination therapy in pulmonary arterial hypertension: new developments

N. Galiè, L. Negro, G. Simonneau
European Respiratory Review 2009 18: 148-153; DOI: 10.1183/09059180.00003809
N. Galiè
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L. Negro
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G. Simonneau
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  • FIGURE 1.
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    FIGURE 1.

    Reduction in mean pulmonary vascular resistance (PVR) in 37 subjects following acute sildenafil administration to ongoing bosentan therapy in the COMPASS-1 (Haemodynamic effects of a single dose of sildenafil in symptomatic patients on bosentan treatment for pulmonary arterial hypertension) study at baseline-2 (mean 889, 95% CI 656–1,121) and 60 min after sildenafil (mean 778, 95% CI 561–995) (per-protocol set). Mean per cent change -15%; 95% CI -21– -10; p<0.0001. Data taken from [20].

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    FIGURE 2.

    The effect of sequential addition of sildenafil to first-line epoprostenol on exercise capacity measured using 6-min walk distance (6MWD) in the PACES clinical trial (Pulmonary Arterial Hypertension Combination Study of Epoprostenol and Sildenafil). ▪: epoprostenol plus sildenafil; •: epoprostenol plus placebo. #: placebo-adjusted increase of 28.8 m, 95% CI 13.9–43.8; p<0.001. Reproduced from [22] with permission of the publisher.

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    FIGURE 3.

    Kaplan–Meier survival estimates for goal-oriented therapy compared with historical treatment and expected survival. ––––: treatment group, 2002–2004; ········: historical control group, 1999–2001; ▪: expected survival. Treatment group versus historical control group, p = 0.011; treatment group versus expected survival, p<0.001 for all time-points. Reproduced from [7] with permission from the publisher.

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    FIGURE 4.

    An example of an algorithm for goal-oriented therapy in pulmonary arterial hypertension (PAH). WHO: World Health Organization; 6MWD: 6-min walk distance; Pra: right atrial pressure; CI: cardiac index; ERA: endothelin receptor antagonist; PDE: phosphodiesterase. Reproduced from [7] permission from the publisher.

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  • Table. 1—

    Summary of combination therapy studies in pulmonary arterial hypertension(PAH) involving small numbers of patients and case reports

    Combination by background therapyPatients nStudy durationAetiology of PAHKey findings: combination versus baseline therapy alone/placeboRef.
    Prostanoids
        Epoprostenol + iloprost8Single doseIPAHSignificant improvements in mean Ppa, CI, Sv,O2, and Pa,O2[23]
        Beraprost + iloprost2312 monthsPAH-CHDImprovement in 6MWD and RVSP[24]
        Beraprost/iloprost + bosentan2016 monthsIPAHImprovement in 6MWD[25]
    1613.5 monthsIPAH, thromboembolicSignificant improvement in 6MWD and Tei index[26]
        Epoprostenol + bosentan81 yrIPAHReduction in epoprostenol dose and side-effectsDiscontinuation of epoprostenol and stabilisation of haemodynamics for ≤1 yr in three patients[27]
    81 yrIPAHSignificant decrease in mean PpaDelay in disease progression[28]
        Iloprost + bosentan912 weeksNot givenSignificant improvement in exercise capacity[29]
        Treprostinil + bosentan19∼3 yrsNot givenSignificant additional improvement in Ppa, 6MWD and Borg dyspnoea scale[30]
        Epoprostenol + sildenafil53 monthsIPAHImprovement in mean Pra and WHO functional class[31]
    35 monthsIPAH, PAH-CHDReduced mean Ppa and PVRIncreased 6MWD[32]
    32 hIPAHSignificant decrease in mean Ppa and PVR[33]
        Iloprost + sildenafil303 hIPAH, CTEPHIncreased vasodilation[34]
    149–12 monthsPAH unresponsive to iloprostImproved 6MWD, haemodynamics and WHO functional class[35]
        Prostanoids + sildenafil202 yrsNot givenSignificant improvement in WHO functional class and signs of right heart failureIncrease in 6MWD after 1- and 2-yr follow-up[36]
    4Not givenIPAH, PAH-CTDImprovements in 6MWD, Ppa and dyspnoea[37]
    Bosentan + sildenafil912 monthsIPAHImprovements in 6MWD and CPET[38]
    3≤24 monthsIPAHImprovement in functional capacity, BNP[39]
    111.1 yrsIPAH, PAH-CTDImprovement in WHO functional class, 6MWD and mean Ppa[40]
    253 monthsIPAH, PAH-SScImproved WHO functional class and 6MWD in IPAH but not in PAH-SSc[41]
    106 monthsIPAH, PAH-CTDIncrease in 6MWD[42]
    Bosentan + prostanoids353 monthsIPAHImprovements in 6MWD, CI, mean Ppa and WHO functional class[43]
    Bosentan + iloprost4012 weeksIPAHNo effect on 6MWD[44]
    6712 weeksIPAH, APAHSignificant increase in 6MWD and WHO functional classDelay in time to clinical worseningImprovements in mean Ppa and PVR[16]
    Sildenafil + beraprost11 monthPAH-SScDecrease in Ppa and PVRImprovements in 6MWD and WHO functional class[45]
    • IPAH: idiopathic PAH; Ppa: pulmonary arterial pressure; CI: cardiac index; Sv,O2: mixed venous oxygen saturation; Pa,O2: arterial oxygen tension; CHD: congenital heart disease; 6MWD: 6-min walk distance; RVSP: right ventricular systolic pressure; Pra: right arterial pressure; WHO: World Health Organization; PVR: pulmonary vascular resistance; CTEPH: chronic thromboembolic pulmonary hypertension; CTD: connective tissue disease; CPET: cardiopulmonary exercise testing; BNP: brain natriuretic peptide; SSc: systemic sclerosis; APAH: associated PAH.

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The use of combination therapy in pulmonary arterial hypertension: new developments
N. Galiè, L. Negro, G. Simonneau
European Respiratory Review Sep 2009, 18 (113) 148-153; DOI: 10.1183/09059180.00003809

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The use of combination therapy in pulmonary arterial hypertension: new developments
N. Galiè, L. Negro, G. Simonneau
European Respiratory Review Sep 2009, 18 (113) 148-153; DOI: 10.1183/09059180.00003809
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  • Article
    • Abstract
    • RATIONALE FOR COMBINATION THERAPY
    • CURRENT DATA ON COMBINATION THERAPY
    • WHEN SHOULD COMBINATION THERAPY BE CONSIDERED?
    • CONCLUSIONS
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