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Survival with first-line bosentan in patients with primary pulmonary hypertension

V. V. McLaughlin, O. Sitbon, D. B. Badesch, R. J. Barst, C. Black, N. Galiè, M. Rainisio, G. Simonneau, L. J. Rubin
European Respiratory Journal 2005 25: 244-249; DOI: 10.1183/09031936.05.00054804
V. V. McLaughlin
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O. Sitbon
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D. B. Badesch
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R. J. Barst
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C. Black
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N. Galiè
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M. Rainisio
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G. Simonneau
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L. J. Rubin
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  • Fig. 1—
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    Fig. 1—

    Kaplan-Meier estimates with 99.9% confidence intervals and predicted survival using the D'Alonzo equation 9. The 99.9% confidence intervals of the Kaplan-Meier estimates do not approach the predicted survival, demonstrating a significant difference between the two curves. ----: observed survival; ––: predicted survival.

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    Fig. 2—

    Vital status and treatment at 12 and 24 months of follow-up. All 169 patients were followed for 12 months; 132 patients were followed for at least 24 months. Percentages are based on the number of patients followed. Embedded Image: dead; └: alive, not on bosentan; ┘: alive, on bosentan and other treatments; □: alive, on bosentan alone.

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    Fig. 3—

    Kaplan-Meier estimates of time to the first elevation of hepatic transaminases to greater than three times the upper limit of normal. About 90% of the cases of elevated liver transaminases occurred during the first 26 weeks of treatment. ALT/AST: alanine aminotransferase/aspartate aminotransferase. Patients at risk: 168, 139, 111, 50, 11, 0 at 0, 30, 60, 90, 120 and 150 weeks from treatment start, respectively.

Tables

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  • Table. 1—

    1 Demographics and baseline characteristics

    Bosentan-treated PPH patientsNIH Registry PPH patients#
    Subjects n169187
    Sex male/female %21/7937/63
    Age yrs
     Mean±sd46±1636±15
     Range13–801–81
    WHO functional class %
     I/II1/8¶0/29
     III/IV82/971
    Time from diagnosis months+
     Mean±sd32±41
     Range0.3–326
    Haemodynamics mean±sd+
     Cardiac index L·min−1·m−22.35±0.802.3±0.9
     PVR Wood units12.9±8.4
     mPAP mmHg57.1±16.060±18
     mRAP mmHg10.1±5.99.7±6.3
    Walk test m
     Mean±sd345±87
    • PPH: primary pulmonary hypertension; NIH: National Institutes of Health; WHO: World Health Organization; PVR: pulmonary vascular pressure; mPAP: mean pulmonary arterial pressure; mRAP: mean right atrial pressure. #: data from Rich et al. 10 provided for context, NIH Registry data were not directly used in any analysis; ¶: all patients entered the pivotal studies in functional class III or IV, but 15 (9%) patients in the placebo group improved before being switched to bosentan in the extension study; +: time from diagnosis and haemodynamic data were available for 157–169 bosentan-treated patients, and for most of these patients were available only at the start of the placebo-controlled study.

  • Table. 2—

    2 Outcomes

    Bosentan-treated PPH patients
    Subjects n169
    Duration of observation for survival
     Mean±sd2.1±0.5 yrs
     Range0.1–3.3 yrs
    Patients lost to follow-up1
    Lung transplantations3
    Deaths n (%)20 (12)#
    Transfers to/additions of prostanoids or alternative oral PPH therapies n (%)39 (23)¶
     Patients who received alternative treatment19
     in addition to bosentan
    Discontinuations of bosentan without other event or treatment4
    • PPH: primary pulmonary hypertension. #: includes the one patient lost to follow-up; ¶: includes the one patient lost to follow-up and three patients with a period of unknown treatment.

  • Table. 3—

    3 Kaplan-Meier (K-M) estimates and predicted survival using the D'Alonzo equation 9

    Time months061218243036
    K-M estimates %10098.896.490.588.585.685.6
    Predicted survival %76.369.262.857.352.448.2
    Patients at risk n1691671631531132316
  • Table. 4—

    4 Prognostic factors for mortality

    Dichotomy/medianHazard ratio95% Confidence limits
    Subjects n169
    WHO functional classIV3.21.1–9.7
    Cardiac index L·min·m−2≤2.251.50.6–3.6
    mPAP mmHg≤541.40.6–3.6
    mRAP mmHg>8.51.90.7–4.8
    6MWT m≤3584.01.3–12.2
    • WHO: World Health Organization; mPAP: mean pulmonary arterial pressure; mRAP: mean right atrial pressure; 6MWT: 6-min walk test.

  • Table. 5—

    5 Adverse events noted by>10% of patients

    Body system/adverse eventsn%
    Aggravated pulmonary hypertension5633.1
    Headache5331.4
    Upper respiratory tract infection3621.3
    Chest pain3218.9
    Dizziness (except vertigo)2917.2
    Dyspnoea2716.0
    Flushing2414.2
    Nasopharyngitis2414.2
    Diarrhoea2313.6
    Syncope2313.6
    Abnormal hepatic function2213.0
    Bronchitis2011.8
    Nausea2011.8
    Cardiac failure1911.2
    Leg oedema1911.2
    Cough1810.7
    Pain in limb1810.7
    Arthralgia1710.1
    Palpitations1710.1
    • Adverse events were collected from the 169 patients during adverse event reporting periods only. The mean duration of observation for adverse events was 78±28 weeks (±sd, median 77 weeks).

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Survival with first-line bosentan in patients with primary pulmonary hypertension
V. V. McLaughlin, O. Sitbon, D. B. Badesch, R. J. Barst, C. Black, N. Galiè, M. Rainisio, G. Simonneau, L. J. Rubin
European Respiratory Journal Feb 2005, 25 (2) 244-249; DOI: 10.1183/09031936.05.00054804

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Survival with first-line bosentan in patients with primary pulmonary hypertension
V. V. McLaughlin, O. Sitbon, D. B. Badesch, R. J. Barst, C. Black, N. Galiè, M. Rainisio, G. Simonneau, L. J. Rubin
European Respiratory Journal Feb 2005, 25 (2) 244-249; DOI: 10.1183/09031936.05.00054804
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