Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary
embolism (PE) with an estimated incidence of 0.5 to
1.5% in the Netherlands, depending on the aetiology of
the PE. The underlying pathophysiological mechanism
is largely unknown and may be caused by (recurrent) emboli or primarily by a characteristic arteriopathy of
the pulmonary arteries. Patients with CTEPH present with nonspecific symptoms predominantly caused by
right heart failure and up to 40% have no prior history of
venous thromboembolism (VTE). The diagnostic approach of CTE PH aims at assessing the location and extent of the embolic obstruction to establish the operability and prognosis of the patients. A heart catheterisation for invasive pressure measurements is obligatory for the final diagnosis. CTEPH is associated with a poor prognosis if left untreated. The preferred treatment is pulmonary endarterectomy. In certain patients with inoperable disease or with persistent or recurrent pulmonary hypertension after surgery, pharmacotherapy might be beneficial.