BACKGROUND: Antiproliferative strategies have emerged as a potential therapeutic option for @entity120 ( @entity120 ). OBJECTIVE: To evaluate the long-term efficacy and safety of @entity2213 . METHODS: This is an observational study of 15 @entity1 with idiopathic @entity120 (n = 13) or @entity120 associated with connective tissue disease (n = 2) treated off-label with @entity2213 400 mg daily. @entity120 -specific therapy was established in all @entity1 (triple therapy in 10, dual therapy in 3, and monotherapy in 2 @entity1 ). RESULTS: After 6 months, improvement in hemodynamics (p < 0.01), functional class (p = 0.035), and quality of life (p = 0.005) was observed. After a median follow-up of 37 months, there was a sustained improvement in functional class (p = 0.032), quality of life (p = 0.019), and echocardiographic parameters of right ventricular function (p < 0.05). Three @entity1 (20%) presented with completely normal echocardiography, absent @entity997 , and normal pro-brain natriuretic peptide levels, indicative of 'hemodynamic remission'. Of note, however, only 1 case was assessed by invasive hemodynamics. The overall 1- and 3-year survival was 100 and 90%, respectively. Two @entity1 experienced a @entity3439 ( @entity3439 ), which in both cases resolved without sequelae. After careful consultation of the potential risks and benefits, all @entity1 as well as a safety cohort of 9 subsequent cases decided to continue the @entity2213 therapy. After adjusting the target international normalized ratio (INR) to around 2.0, no further cases of @entity3439 occurred during 50 @entity1 -years. CONCLUSIONS: Long-term treatment with @entity2213 may improve the functional class and quality of life. Single cases might even attain hemodynamic remission. The occurrence of 5% @entity3439 per @entity1 -years is concerning. However, adjusting the INR to around 2.0 might obviate this complication.    2015 S. Karger AG, Basel.

Efficacy and Safety of Long-Term XXXX Therapy for Pulmonary Arterial Hypertension.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity3439 :: ('MESH:D006408', 'Disease') :: ['subdural hematoma', 'SDH'] @entity120 :: ('MESH:D006976', 'Disease') :: ['PAH', 'pulmonary arterial hypertension', 'Pulmonary hypertension'] @entity997 :: ('MESH:D014262', 'Disease') :: ['tricuspid regurgitation'] @entity2213 :: ('MESH:C097613', 'Chemical') :: ['imatinib']
@entity2213 :: (MESH:C097613,Chemical) :: ['Imatinib']
