Adempas used to Treat PAH and CTEPH

Adempas: a soluble guanylate cyclase stimulator used to treat PAH and chronic thromboembolic pulmonary hypertension

Soluble guanylate cyclase stimulators work by stimulating an enzyme inside cells called soluble guanylate cyclace.  By increasing the activity of this enzyme, there is an increase in the production of cyclic GMP, which in turn leads to relaxation of the pulmonary arteries and improvements in pulmonary hypertension.  The pathway by which soluble guanylate cyclase stimulators work is similar to the way in which PDEV inhibitors work but the action is at an earlier point in the cascade.  The FDA (Food and Drug Administration) recently approved the first soluble guanylate cyclase stimulator to treat pulmonary arterial hypertension.

Adempas (Riociguat) an oral treatment for PAH and CTEPH

Adempas is the first in its class of medications (soluble guanylate cyclase stimulators) approved by the FDA to treat PAH.   Two studies have been published recently in the New England Journal of Medicine that showed that Adempas improved exercise capacity and delayed clinical worsening in patients with PAH and chronic thromboembolic pulmonary hypertension.  The most common side effects were head ache, stomach symptoms, swelling of the legs and low blood pressure.  The medication is taken orally and the dose is gradually increased from to a maximum of 2.5mg three times daily.  This medicine is a teratogen meaning it can cause harm to a developing fetus.  Women must use two methods of reliable birth control and have monthly pregnancy tests while taking the medication.  All patients must complete the Adempas Enrollment Form prior to receiving the medication.

Adempas is available through a small network of specialty pharmacies.  Nursing visits from a nurse with the specialty pharmacy are required.  It is important not to start this medication until the nurse from the specialty pharmacy has measured the patient’s blood pressure and instructed the patient to do so. It is important to take every dose of this medication.  If a patient misses multiple doses they should contact their physicians office as they may have to restart the drug at a lower dose.