is one of five types of PH designated by the World Health Organization as Group 4.
It is estimated that 500 to 2500 patients develop CTEPH
every year in the U.
PHA has created a patient-centered page on their website dedicated to CTEPH
awareness and education, www.
Riociguat is the first drug therapy that has shown clinical efficacy in the treatment of inoperable CTEPH
patients, or patients with persistent or recurrent CTEPH
Each year, in the US, about 600,000 persons have an acute PE, and it is estimated that there are between 500 and 2,500 new cases of CTEPH
diagnosed each year.
The FDA s approval is a key milestone in our efforts to provide patients and physicians with a much-needed new treatment option for this rare, serious and potentially fatal disorder: It is the first drug to be approved for inoperable CTEPH
or for persistent or recurrent disease after surgery, and it is also an important new class of treatment for patients with PAH, said Dr.
The drug is approved in Canada for both the above-mentioned indications and in Switzerland and Japan for CTEPH
PAH and CTEPH
are both life-threatening forms of pulmonary hypertension that cause significantly increased pressure in the pulmonary arteries.
Riociguat is the first drug to demonstrate efficacy in two distinct forms of pulmonary hypertension, namely PAH and inoperable CTEPH
riociguat) tablets for: (i) the treatment of adults with persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH
) (WHO* Group 4) after surgical treatment or inoperable CTEPH
to improve exercise capacity and WHO functional class; and (ii) the treatment of adults with pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity, improve WHO functional class and delay clinical worsening.
is a progressive and life-threatening disease in which it is believed that blood clots of pulmonary vessels gradually lead to an increased pressure in the pulmonary arteries, resulting in an overload of the right heart.
To date, no approved pharmacological therapy exists for CTEPH
and as a result there is an urgent unmet medical need for patients who are unable to undergo surgery.
may evolve after prior episodes of acute pulmonary embolism, but the pathogenesis is not yet completely understood.
The results of the CHEST-2 trial support the positive data of the pivotal CHEST-1 trial, showing long-term safety and sustained clinical benefits in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH
) or with persistent or recurrent CTEPH
after a surgical procedure called pulmonary endarterectomy (PEA).
Bayer has also submitted a regulatory application for the CTEPH
indication of riociguat, in Japan.