Figures
- 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].
- 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.
- 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.
- 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.
Tables
- Table. 1—
Summary of combination therapy studies in pulmonary arterial hypertension(PAH) involving small numbers of patients and case reports
Combination by background therapy Patients n Study duration Aetiology of PAH Key findings: combination versus baseline therapy alone/placebo Ref. Prostanoids Epoprostenol + iloprost 8 Single dose IPAH Significant improvements in mean Ppa, CI, Sv,O2, and Pa,O2 [23] Beraprost + iloprost 23 12 months PAH-CHD Improvement in 6MWD and RVSP [24] Beraprost/iloprost + bosentan 20 16 months IPAH Improvement in 6MWD [25] 16 13.5 months IPAH, thromboembolic Significant improvement in 6MWD and Tei index [26] Epoprostenol + bosentan 8 1 yr IPAH Reduction in epoprostenol dose and side-effectsDiscontinuation of epoprostenol and stabilisation of haemodynamics for ≤1 yr in three patients [27] 8 1 yr IPAH Significant decrease in mean PpaDelay in disease progression [28] Iloprost + bosentan 9 12 weeks Not given Significant improvement in exercise capacity [29] Treprostinil + bosentan 19 ∼3 yrs Not given Significant additional improvement in Ppa, 6MWD and Borg dyspnoea scale [30] Epoprostenol + sildenafil 5 3 months IPAH Improvement in mean Pra and WHO functional class [31] 3 5 months IPAH, PAH-CHD Reduced mean Ppa and PVRIncreased 6MWD [32] 3 2 h IPAH Significant decrease in mean Ppa and PVR [33] Iloprost + sildenafil 30 3 h IPAH, CTEPH Increased vasodilation [34] 14 9–12 months PAH unresponsive to iloprost Improved 6MWD, haemodynamics and WHO functional class [35] Prostanoids + sildenafil 20 2 yrs Not given Significant improvement in WHO functional class and signs of right heart failureIncrease in 6MWD after 1- and 2-yr follow-up [36] 4 Not given IPAH, PAH-CTD Improvements in 6MWD, Ppa and dyspnoea [37] Bosentan + sildenafil 9 12 months IPAH Improvements in 6MWD and CPET [38] 3 ≤24 months IPAH Improvement in functional capacity, BNP [39] 11 1.1 yrs IPAH, PAH-CTD Improvement in WHO functional class, 6MWD and mean Ppa [40] 25 3 months IPAH, PAH-SSc Improved WHO functional class and 6MWD in IPAH but not in PAH-SSc [41] 10 6 months IPAH, PAH-CTD Increase in 6MWD [42] Bosentan + prostanoids 35 3 months IPAH Improvements in 6MWD, CI, mean Ppa and WHO functional class [43] Bosentan + iloprost 40 12 weeks IPAH No effect on 6MWD [44] 67 12 weeks IPAH, APAH Significant increase in 6MWD and WHO functional classDelay in time to clinical worseningImprovements in mean Ppa and PVR [16] Sildenafil + beraprost 1 1 month PAH-SSc Decrease 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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