Long-Term Outcome of Bosentan Treatment in Idiopathic Pulmonary Arterial Hypertension and Pulmonary Arterial Hypertension Associated with the Scleroderma Spectrum of Diseases

First-line bosentan monotherapy is associated with long-term improvement in functional class and good overall survival in patients with WHO class III IPAH. Most APAH-SSD patients experienced stability or decline in functional class and tended to have a higher mortality.