Bosentan for Severe Mitral Valve Dysfunction
Bosentan for Severe Mitral Valve Dysfunction
Vasoconstrictive signaling via endothelin receptors is not limited to primary pulmonary
 arterial hypertension, but has also been documented in secondary pulmonary hypertension due
 to congestive heart failure, including cardiac valve disease. The investigators aim to
 examine the clinical and physiologic effects of bosentan therapy in patients with secondary
 pulmonary hypertension due to severe, inoperable cardiac valve disease, using a
 single-center, prospective, open-label, non-randomized study of oral bosentan in outpatients
 with severe mitral stenosis due to childhood rheumatoid fever. Primary end-point will be
 exercise capacity at six months determined by six-minute walking distance and cardiopulmonary
 exercise testing. Secondary end-points will be symptomatic relief, echocardiographic left
 ventricular function and pulmonary pressure, serum pro-brain natriuretic peptide, and adverse
 events at six months.
Inclusion Criteria:

 -  Outpatient

 -  > 60 Yrs Old

 -  < 85 Yrs Old

 -  Stable disease

 -  Congestive heart failure NYHA IIIB/V

 -  Inoperable mitral stenosis due to childhood rheumatoid fever

 -  Mean pulmonary artery pressure > 40 cm H2O

 Exclusion Criteria:

 -  Prior treatment with endothelin receptor antagonist(s)

 -  Hospitalization (exacerbation)

 -  Cardiac valve surgery