The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study

¹ Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge, UK
² Antoine Béclère Hopital, Paris-Sud University, Clamart, France
³ Dept of Cardiology, Medical University of Vienna, Vienna, Austria

^* To whom correspondence should be addressed. E-mail: Joanna.pepkazaba{at}papworth.nhs.uk.

	Abstract

The treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA). However, many patients develop a severe progressive small vessel pulmonary arteriopathy which is inaccessible to surgical intervention and is associated with poor survival. The purpose of the study was to evaluate the medium term efficacy and safety of the dual endothelin receptor antagonist, bosentan, in inoperable CTEPH.

47 patients with inoperable CTEPH (distal disease or persistent pulmonary hypertension following PEA) underwent evaluation after one year of bosentan therapy. Outcomes included assessment of six minute walk test (6MWT), haemodynamics and WHO functional classification. Monitoring of serious adverse effects and changes in therapy was undertaken.

Patients showed sustaining improvements in 6MWT (49m +/- 8m), functional classification, cardiac index (+0.2 L·min^-1·m^-2±0.07) and total pulmonary resistance (-139 dyn·s·cm^-5±42). Those patients with persisting pulmonary hypertension following PEA showed the greatest improvement. One year survival was 96%, bosentan was well tolerated with only one patient developing derranged liver function.

Although all patients with CTEPH should be considered for PEA, bosentan provides an alternative medical therapy to improve function and delay the progression of this devastating disease in those in who surgery is not suitable.

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