Margita Belicová
According to the current definition, chronic thromboembolic pulmonary hypertension is a symptomatic pulmonary hypertension with persistent defect of perfusion despite three month appropriate anticoagulant therapy, with a clear mechanical component and varying pulmonary hypertensive arteriopathy of small distal vessels. The main symptom of the disease is an exertional dyspnoea. In suspected patients with pulmonary hypertension the obstructive nature may be revealed by perfusion lung scan and computed tomography may be useful to rule out confounding disorders. Pulmonary angiography is the gold standard for defining the pulmonary vascular anatomy and to identify whether any obstruction is present. Treatment is surgical, thromendarterectomy. In inoperable cases, alternative might be specific novel therapies of pulmonary arterial hypertension, including prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase-5 inhibitors. Lifelong oral anticoagulants are indicated in all these patients.