OBJECTIVE: To evaluate the short- and long-term effects of intravenous @entity4770 in @entity1 with @entity120 ( @entity120 ) associated with @entity785 ( @entity785 ). METHODS: Sixteen @entity1 with @entity785 -associated @entity120 and New York Heart Association (NYHA) class III or IV symptomatology underwent right heart catheterization for determination of baseline hemodynamic values. Vasoreactivity was assessed with either inhaled @entity842 or intravenous @entity859 . After a medication washout period, all @entity1 received intravenous @entity4770 in incrementally increasing doses; tolerance was assessed according to symptoms and hemodynamic findings at each dose increment and at the conclusion of the medication trial. Once a stable medication regimen was established, @entity1 were discharged and followed up as outpatients for assessment of symptoms and exercise tolerance as measured by change in the NYHA class. Repeat hemodynamic testing was performed in 4 @entity1 at 1 year and in 2 @entity1 at 2 years of treatment. RESULTS: Therapeutic response to @entity4770 , defined by a reduction in the pulmonary vascular resistance of > or =25%, was achieved in the short-term treatment period in 13 of 16 @entity1 (81.3%). Improvement in symptoms and exercise tolerance occurred in all @entity1 , and a significant short-term hemodynamic response was observed. Followup hemodynamic tests revealed persistent favorable responses in all 4 of the @entity1 studied. CONCLUSION: Most @entity1 with @entity120 secondary to @entity785 manifest favorable hemodynamic responses to @entity4770 in the short term. Long-term @entity4770 was generally well tolerated and provides a potential therapeutic option for @entity1 with @entity120 secondary to @entity785 .

@entity785 -associated @entity120 : short- and long-term effects of @entity4770 ( XXXX ).

@entity1 :: ('9606', 'Species') :: ['patients'] @entity859 :: ('MESH:D000241', 'Chemical') :: ['adenosine'] @entity120 :: ('MESH:D006976', 'Disease') :: ['pulmonary hypertension', 'PH'] @entity4770 :: ('MESH:D011464', 'Chemical') :: ['epoprostenol'] @entity842 :: ('MESH:D009569', 'Chemical') :: ['nitric oxide'] @entity785 :: ('MESH:D012595', 'Disease') :: ['systemic sclerosis', 'SSc']
@entity4770 :: (MESH:D011464,Chemical) :: ['prostacyclin']
