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10.1136/heartjnl-2015-307832

http://scihub22266oqcxt.onion/10.1136/heartjnl-2015-307832
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suck abstract from ncbi


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pmid26135803      Heart 2015 ; 101 (22): 1792-9
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  • Riociguat for pulmonary arterial hypertension associated with congenital heart disease #MMPMID26135803
  • Rosenkranz S; Ghofrani HA; Beghetti M; Ivy D; Frey R; Fritsch A; Weimann G; Saleh S; Apitz C
  • Heart 2015[Nov]; 101 (22): 1792-9 PMID26135803show ga
  • Objective: The Pulmonary Arterial hyperTENsion sGC-stimulator Trial-1 (PATENT-1) was a randomised, double-blind, placebo-controlled phase III trial evaluating riociguat in patients with pulmonary arterial hypertension (PAH). PATENT-2 was an open-label long-term extension to PATENT-1. Here, we explore the efficacy and safety of riociguat in the subgroup of patients with persistent/recurrent PAH after correction of congenital heart disease (PAH-CHD) from the PATENT studies. Methods: In PATENT-1, patients received riociguat (maximum 2.5 or 1.5?mg three times daily) or placebo for 12?weeks; efficacy assessments included change from baseline to study end in 6-min walking distance (6MWD; primary), pulmonary vascular resistance (PVR), N-terminal of the prohormone of brain natriuretic peptide (NT-proBNP), WHO functional class (WHO FC) and time to clinical worsening. In PATENT-2, eligible patients from PATENT-1 received long-term riociguat (maximum 2.5?mg three times daily); the primary assessment was safety and tolerability. All PAH-CHD patients had a corrected cardiac defect. Results: In PATENT-1, riociguat increased mean±SD 6MWD from baseline to week 12 by 39±60?m in patients with PAH-CHD versus 0±42?m for placebo. Riociguat also improved several secondary variables versus placebo, including PVR (?250±410 vs ?66±632?dyn·s/cm5), NT-proBNP (?164±317 vs ?46±697?pg/mL) and WHO FC (21%/79%/0% vs 8%/83%/8% improved/stabilised/worsened). One patient experienced clinical worsening (riociguat 1.5?mg group). Riociguat was well tolerated. In PATENT-2, riociguat showed sustained efficacy and tolerability in patients with PAH-CHD at 2?years. Conclusions: Riociguat was well tolerated in patients with PAH-CHD and improved clinical outcomes including 6MWD, PVR, WHO FC and NT-proBNP. Trial registration number: The clinical trials numbers are NCT00810693 for PATENT-1 and NCT00863681 for PATENT-2.
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