Background and objective: Chronic thromboembolic pulmonary hypertension (CTEPH) has a dismal prognosis when there are no central pulmonary thrombi amenable to surgical thromboendarterectomy. Pulmonary vasodilators could be useful in this setting. Initial experience with bosentan in a small group of patients with CTEPH has shown favourable results on the short term (3 to 6 months), but long-term effects remain unknown.
Patients and method: We retrospectively describe the effects of bosentan in 6 CTEPH patients with a mean follow-up period of 15 months (range, 8-26).
Results: At 3-month follow-up, all patients had experienced clinical improvement, with a statistical trend towards reduced pulmonary vascular resistance [1,008 (624) dyn/sec/cm-5 versus 768 (392), p = 0.07]. Clinical improvement persisted on the long-term, [baseline NYHA functional class 3.0 (0.4) versus 2.0 (0) at the last follow-up visit, p < 0.01]. Six-minute walk-test results [baseline 230 (124) meters versus a 313 (70) at 1 year] and NTproBNP [2,225 (2,079) pg/ml versus a 1,056 (1,104) at 1 year] were also consistent with persistent beneficial effect.
Conclusions: Bosentan seemed to provide long-term benefits in this small series of patients with CTEPH.