Targeted pulmonary arterial hypertension (PAH) treatment may attenuate hemodynamic progression in patients with systemic sclerosis who have borderline abnormal pulmonary hemodynamics.
Targeted pulmonary arterial hypertension (PAH) treatment may attenuate hemodynamic progression in patients with systemic sclerosis who have borderline abnormal pulmonary hemodynamics. Resting and exercise pulmonary artery pressure (PAP) may increase significantly within 1 year of follow-up; treatment with bosentan may be safe and effective.
In an exploratory pilot study conducted by Kovacs and colleagues, patients with borderline PAH underwent right heart catheterization at baseline, after a 12-month observation period, and after 6 months of bosentan therapy. They compared changes in mean PAP at 50W during the observation and therapy periods.
Mean PAP at rest, at 50W, and during maximal exercise increased significantly during the observation period and tended to decrease during the treatment period. The changes that occurred during these periods were significantly different. The changes in resting pulmonary vascular resistance also were significantly different during the periods. Changes in resting pulmonary arterial wedge pressure were not significantly different between the periods, in spite of the significant increase during the observation period. No relevant adverse effects were reported.
The authors noted that randomized controlled trials, including assessment of potential changes in left ventricular function, are warranted to confirm the findings of this hypothesis-generating pilot study.