Stephan Moll, MD writes…
On Oct 8th, 2013 the FDA approved a new drug to treat severe pulmonary hypertension. The drug is called Adempas® (riociguat).
Blood clots in the lung (PE) can sometimes cause left-over symtpoms, such as shortness of breath, decreased exercise ability, or chest discomfort, if the clots do not resolve completely. Damage to the lung may occur. If the damage is severe, then this is called pulmonary hypertension. It has also been given the name CTEPH, which stands for chronic thromboembolic pulmonary hypertension. About 3.8 % of patients (1 of 25) develop CTEPH after a PE. It is a potentially life threatening condition, leading to heart failure. Patients who have had a PE and who have persistent or recurrent symptoms should be evaluated by their doctor for sing of CTEPH, particularly if the PE was extensive. Such an evalaluation should be considered several months after the PE, if the patient fails to get back to his/her baseline or feeling well.
The new drug works by relaxing the blood vessels in the lung, resulting in improved extercise abilities for the patient. It is taken as a tablet three times a day. The FDA approval came based on two important clinical studies published in 2013 [ref 1,2]. The FDA release is here. The drug should /will be prescribed by physicians in pulmonary hypertension clinics. Such specialized pulmonary clinics already exist and there is an additional effort under way to designate appropriate clinics as “Pulmonary Hypertension Comprehensive Care Centers” (details here) – to be launched in 2014. This is being coordinated the Pulmonary Hypertension Association (web link here).
- Ghofrani HA et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013;369:319-329.
- Ghofrani HA et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013;369:330-40.
I have no financial conflict of interest.
Last updated: Oct 16th, 2013