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Copyright ©ERS Journals Ltd 2006
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 Hospital, Paris-Sud University, Clamart, France. 3 Dept of Cardiology, Medical University of Vienna, Vienna, Austria.
CORRESPONDENCE: R. J. Hughes, Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridgeshire, CB3 8RE, UK, Fax: 44 1480831068. E-mail: rodneyhughes{at}onetel.com
Keywords: Bosentan, chronic thromboembolic pulmonary hypertension, endothelin, pulmonary hypertension, thromboembolic
Received: November 17, 2005
Accepted March 28, 2006
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 that is inaccessible to surgical intervention and is associated with poor survival. The purpose of the present study was to evaluate the medium-term efficacy and safety of the dual endothelin receptor antagonist, bosentan, in inoperable CTEPH.
Forty-seven patients with inoperable CTEPH (distal disease or persistent pulmonary hypertension following PEA) underwent evaluation after 1 yr of bosentan therapy. Outcomes included assessment of 6-min walk test (6MWT), haemodynamics and World Health Organization functional classification. Monitoring of serious adverse effects and changes in therapy was undertaken.
Patients showed sustained improvements in 6MWT (49±8 m), functional classification, cardiac index (+0.2±0.07 L·min-1·m-2) and total pulmonary resistance (-139±42 dyn·s·cm-5). Those patients with persisting pulmonary hypertension following PEA showed the greatest improvement. One-yr survival was 96%, and bosentan was well tolerated with only one patient developing deranged liver function.
Although all patients with chronic thromboembolic pulmonary hypertension should be considered for pulmonary endarterectomy, bosentan provides an alternative medical therapy to improve function and delay the progression of this devastating disease in those in whom surgery is not suitable.
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