PURPOSE: To describe the response to treatment and side-effects of pulmonary hypertension (PH) patients treated with bosentan.
METHODS: Retrospective analysis of consecutive PH patients in a single referral center.
RESULTS: Forty-seven PH patients were treated with bosentan. Five patients were excluded due to incomplete data. Of the remaining 42 patients, mean age was 59 ± 16 years. Thirty-one (74%) were women. The diagnostic categories were: primary pulmonary hypertension (PPH) 12 or 29%, connective tissue disease 10 or 24%, and PH out of proportion to co-existing lung disease 7 (17%). Most patients were WHO class 3 or 4 (22 or 52% and 15 or 36% respectively). Of the 37 (88%) patients with right heart catheterization prior to treatment, 27 had an acute vasodilator trial with epoprostenol and 18 were positive. Mean pulmonary artery systolic pressure (PASP) by echocardiogram prior to treatment was 80 ± 23. After at least 6-8 weeks of treatment, the PASP remained unchanged at 80 ± 24. Only 10 (24%) patients had clinical improvement. The clinical response rate was similar in PPH. Additional vasodilator therapy was initiated in 22 (52%) patients, including Flolan in 10 patients. Bosentan was discontinued in 21 of 42 patients (50%) usually due to side effects (11 patients). The most common side effect was liver enzyme elevation (9 patients). Overall mortality was high: 9 of 42 (21%). An additional 4 patients (9%) required lung transplant or heart lung transplant.
CONCLUSION: A minority of the patients responded to Bosentan therapy. The frequency of side effects noticed was higher than has been published.
CLINICAL IMPLICATIONS: The low clinical response rate and high drug intolerance may limit the utility of bosentan in patients with severe PH.
DISCLOSURE: Abubakr Bajwa, None.