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DOI: 10.1055/s-0035-1555984
Riociguat Treatment for Pulmonary Arterial Hypertension (PAH) Associated with Congenital Heart Disease (CHD): A Subgroup Analysis from the PATENT Studies
Introduction: Riociguat has shown beneficial effects in patients with PAH in the Phase III PATENT-1 study and the PATENT-2 extension.
Objective: To investigate the safety and efficacy of riociguat in patients with persistent or recurrent PAH following complete surgical repair of CHD (PAH-CHD) in PATENT-1 and PATENT-2.
Methods: In PATENT-1, patients received either riociguat up to 2.5 mg three times daily (tid), riociguat up to 1.5 mg tid, or placebo for 12 weeks. In PATENT-2, all patients received open-label riociguat up to 2.5 mg tid.
Results: In PATENT-1, 35 patients had PAH-CHD, of whom 33 entered PATENT-2. Riociguat was well tolerated in patients with PAH-CHD and improved several clinical endpoints vs placebo at Week 12 (Table 1; observed values are shown). Improvements in 6-minute walking distance (6MWD) and World Health Organization functional class (WHO FC) persisted for up to 2 years in PATENT-2.
Conclusion: Riociguat is a promising treatment for PAH-CHD patients.
|
Endpoint (data are mean±SD or %) |
Riociguat 2.5 mg-max |
Riociguat 1.5 mg-max |
Placebo |
||||
|---|---|---|---|---|---|---|---|
|
n |
Δ BL |
N |
Δ BL |
n |
Δ BL |
||
|
aImproved/stabilized/worsened. |
|||||||
|
Δ BL, change vs baseline at Week 12. |
|||||||
|
6MWD, m |
13 |
+39 ± 60 |
7 |
+43 ± 54 |
12 |
0 ± 42 |
|
|
PVR, dyn·s·cm−5 |
13 |
−250 ± 410 |
7 |
−126 ± 368 |
11 |
−66 ± 632 |
|
|
NT-proBNP, pg/mL |
12 |
−164 ± 317 |
6 |
−872 ± 1147 |
12 |
−46 ± 697 |
|
|
WHO FC, %a |
14 |
21/79/0 |
7 |
29/71/0 |
12 |
8/83/8 |
|