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 another disease.2,3

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 therapy.

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

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  • 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.