Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 25mmHg. Chronic thromboembolic pulmonary hypertension (CTEPH) is defined as pulmonary hypertension in the presence of an organized thrombus within the pulmonary vascular bed that persists at least 3 months after the onset of anticoagulant therapy.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 PH in that it is potentially curable only with pulmonary endarterectomy (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 Respir J 2015;46:903–75.
  • Piazza G, Goldhaber SZ. N Engl J Med 2011;364:351–60.
  • Tapson V et al. Proc Am Thorac Soc 2006;3:564-7.
  • Pengo V et al. N Engl J Med 2004;350:2257–64.