Chronic thromboembolic pulmonary hypertension (CTEPH)

Active Ingredient: Riociguat

Indication for Riociguat

Population group: only adults (18 years old or older)
Therapeutic intent: Curative procedure

Riociguat is indicated for the treatment of adult patients with WHO Functional Class (FC) II to III with:

  • inoperable CTEPH,
  • persistent or recurrent CTEPH after surgical treatment, to improve exercise capacity.

For this indication, competent medicine agencies globally authorize below treatments:

1 mg 3 times daily for 2 weeks and thereafter individual dose titration every two weeks to a maximum of 2.5 mg 3 times daily

For:

Dosage regimens

Oral, 1 milligrams riociguat, 3 times daily, over the duration of 2 weeks. Afterwards, oral, between 1 milligrams riociguat and 2.5 milligrams riociguat, 3 times daily. The maximum allowed total dose is 7.5 milligrams riociguat daily.

Detailed description

Starting dose

The recommended starting dose is 1 mg 3 times daily for 2 weeks. Doses should be taken 3 times daily approximately 6 to 8 hours apart.

Titration

Dose should be increased by 0.5 mg 3 times daily every two weeks to a maximum of 2.5 mg 3 times daily, if systolic blood pressure is ≥95 mmHg and the patient has no signs or symptoms of hypotension. In some PAH patients, an adequate response on the 6-minute walk distance (6MWD) may be reached at a dose of 1.5 mg 3 times a day. If systolic blood pressure falls below 95 mmHg, the dose should be maintained provided the patient does not show any signs or symptoms of hypotension. If at any time during the up-titration phase systolic blood pressure decreases below 95 mmHg and the patient shows signs or symptoms of hypotension the current dose should be decreased by 0.5 mg 3 times daily.

Maintenance dose

The established individual dose should be maintained unless signs and symptoms of hypotension occur.

The maximum total daily dose is 7.5 mg (i.e., 2.5 mg 3 times daily) for adults patients.

If a dose is missed, treatment should be continued with the next dose as planned.

If not tolerated, dose reduction should be considered at any time.

Treatment discontinuation

In case treatment has to be interrupted for 3 days or more, treatment should be restarted with 1 mg 3 times daily for 2 weeks and continued with the dose titration regimen as described above.

Transitioning between phosphodiesterase-5 (PDE5) inhibitors and riociguat

Sildenafil must be discontinued in adults at least 24 hours prior to administration of riociguat.

Tadalafil must be discontinued at least 48 hours in adults prior to administration of riociguat.

Riociguat must be discontinued in adults at least 24 hours prior to administration of a PDE5 inhibitor.

It is recommended to monitor for signs and symptoms of hypotension after any transition.

Dosage considerations

For patients prone to hypotension, as a precautionary measure, switches between fed and fasted riociguat intake are not recommended because of increased peak plasma levels of riociguat in the fasting compared to the fed state.

Active ingredient

Riociguat

Riociguat is a stimulator of soluble guanylate cyclase (sGC), an enzyme in the cardiopulmonary system and the receptor for nitric oxide (NO). Riociguat restores the NO-sGC-cGMP pathway and leads to increased generation of cGMP.

Read more about Riociguat

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