Epidemiology and management of chronic thromboembolic pulmonary hypertension

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 CTEPH 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.