Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma …

RE Girgis, SC Mathai, JA Krishnan, FM Wigley… - The Journal of heart and …, 2005 - Elsevier
BACKGROUND: Bosentan improves clinical outcomes in pulmonary arterial hypertension
(PAH), particularly in the idiopathic (IPAH) subset. Scant data are available regarding PAH
associated with the scleroderma spectrum of diseases (APAH-SSD). Here we review our
experience with bosentan in these 2 groups. METHODS: Included were all patients at our
center with either IPAH or APAH-SSD in whom bosentan was the first-line, single-agent
therapy with at least 6 months of follow-up. Changes in the World Health Organization …