Chronic Thromboembolic Pulmonary Hypertension, or CTEPH, is a rare, life
threatening form of PH that develops when pulmonary artery branches and mean
pulmonary arterial pressure (mPAP) rises.1 The most commonly reported
symptoms of CTEPH include exercise intolerance, fatigue, and dyspnea. Patients
may subsequently report chest discomfort, syncope, hemoptysis, light-headedness,
or peripheral leg edema.2
Symptoms experienced by CTEPH patients are similar to those of other more
common diseases, like asthma and chronic obstructive pulmonary disease, and
other types of PH. In part because these symptoms are nonspecific, CTEPH is
underdiagnosed and often misdiagnosed as
But CTEPH is unique among the five groups of pulmonary hypertension (PH) in that
it is potentially curable only with pulmonary thromboendarterectomy (PEA)
surgery, which is the treatment choice for patients who
are surgical candidates.2 For patients who are not surgical candidates or who have persistent/recurrent CTEPH after PEA surgery
, there is an approved medical
It is essential that patients suspected of suffering from CTEPH (or any form of PH)
are referred to an expert center. Diagnosis should be made with urgency, as the
disease can progress quickly.4
- Galiè N et al. Eur Heart J 2015:doi:10.1093/eurheart/ehv317.
- Piazza G and Goldhaber SZ. N Engl J Med. 2011;364:351-360.
- Tapson V et al. Proc Am Thorac Soc. 2006;3:564-7.
- Pengo V et al. N Engl J Med 2004;350:2257–264.