Long-term advanced therapy with bosentan improves symptoms and prevents deterioration of inoperable chronic thromboembolic pulmonary hypertension.

AIMS: Chronic thromboembolic pulmonary hypertension (CTEPH) is a devastating disease, and treating patients with CTEPH who are not eligible for pulmonary endarterectomy is particularly challenging. Short-term bosentan or phosphodiesterase type-5-inhibitor treatment can greatly improve symptoms and hemodynamics in these patients; however, the long-term benefits of advanced pulmonary vasodilators are not known. We retrospectively investigated the long-term effects of advanced pharmacological therapy in Japanese patients with inoperable CTEPH. MAIN METHODS: Seven consecutive patients with inoperable CTEPH (five women; mean age, 62.6±6.9 years) treated with bosentan were included. World Health Organization functional class (WHO-FC), hemodynamics, exercise capacity, and plasma B-type natriuretic peptide (BNP) concentration were evaluated at baseline and for more than 2 years. Time to clinical worsening was also examined during long-term follow-up. KEY FINDINGS: WHO-FC improved significantly, from 3.1±0.4 to 2.1±0.4 (p=0.005). Significant improvement was also seen in pulmonary vascular resistance, which decreased from 786.9±300.0 to 352.2±210.7 dynes s cm(-5) (p<0.05). Plasma BNP concentration decreased significantly from 1160.0±971.4 to 305.1±285.9 pg/mL (p<0.05). No patient required hospitalization during the follow-up period (mean, 896±564 days). SIGNIFICANCE: Long-term advanced therapy with bosentan significantly improves symptoms, pulmonary vascular resistance, plasma BNP concentration, and time to clinical worsening in Japanese patients with inoperable CTEPH. We consider bosentan to be an essential treatment for these patients.