Universität zu Köln

Rosenkranz, Stephan, Ghofrani, Hossein-Ardeschir, Beghetti, Maurice, Ivy, Dunbar, Frey, Reiner, Fritsch, Arno, Weimann, Gerrit, Saleh, Soundos and Apitz, Christian (2015). Riociguat for pulmonary arterial hypertension associated with congenital heart disease. Heart, 101 (22). S. 1792 - 1800. LONDON: BMJ PUBLISHING GROUP. ISSN 1468-201X

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Abstract

Objective The Pulmonary Arterial hyperTENsion sGC-stimulator Trial-1 (PATENT-1) was a randomised, doubleblind, 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/cm(5)), 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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Rosenkranz, Stephan UNSPECIFIED UNSPECIFIED UNSPECIFIED Ghofrani, Hossein-Ardeschir UNSPECIFIED UNSPECIFIED UNSPECIFIED Beghetti, Maurice UNSPECIFIED UNSPECIFIED UNSPECIFIED Ivy, Dunbar UNSPECIFIED UNSPECIFIED UNSPECIFIED Frey, Reiner UNSPECIFIED UNSPECIFIED UNSPECIFIED Fritsch, Arno UNSPECIFIED UNSPECIFIED UNSPECIFIED Weimann, Gerrit UNSPECIFIED UNSPECIFIED UNSPECIFIED Saleh, Soundos UNSPECIFIED UNSPECIFIED UNSPECIFIED Apitz, Christian UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-388239
DOI:	10.1136/heartjnl-2015-307832
Journal or Publication Title:	Heart
Volume:	101
Number:	22
Page Range:	S. 1792 - 1800
Date:	2015
Publisher:	BMJ PUBLISHING GROUP
Place of Publication:	LONDON
ISSN:	1468-201X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language GUANYLATE-CYCLASE STIMULATORS; SURVIVAL; PREVALENCE; BOSENTAN; THERAPY; ADULTS; IMPACT Multiple languages Cardiac & Cardiovascular Systems Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/38823