To asses the response of patients with underlying pulmonary arterial hypertension who do not qualify for treatment with bosentan to treatment with bosentan.
Fifteen patients (8 females and 7 males) with underlying pulmonary arterial hypertension who did not qualify for treatment with bosentan [3 with chronic thromboembolic disease (CTED), 2 with congenital and valvular disease (CVD), 3 with interstitial lung disease and severe fibrosis (ILD), and 7 with connective tissue disease and severe fibrosis (CTD+FIB)] where treated with bosentan. Bosentan was started at 62.5 mg PO BID and increased to 125 mg PO BID after 4 weeks. One patient with CTED was started on dual therapy with bosentan and sildenafil. Primary end points where 6-minute walk distance and NYHA functional class. Patients were assessed at baseline, 3 and 6 months.
The overall 6-minute walk distance at baseline, 3 and 6 months (Mean±SE, meters) were 262.2±36.76, 357.4±38.60, and 366.3±44.92 respectively. Individual as well as group data (CTED, CVD, ILD, CTD+FIB) are shown in Figure 1. Overall NYHA class dropped from an average of 3 to 2.2 both at 3 and 6 months. All patients tolerated the treatment well. Two patients required a reduction in dose down to 62.5 mg PO BID.
Patients with underlying pulmonary arterial hypertension that do not qualify for treatment with endothelin receptor antagonists may still benefit from treatment.
More RCT are needed to assess the response to treatment in wider groups of patients with underlying pulmonary arterial hypertension.
Zoheir Bshouty, None.