Interní Med. 2008; 10(9): 392-396

The treatment of chronic pulmonary hypertension

MUDr. Pavel Jansa¹, prof. MUDr. Michael Aschermann DrSc², prof. MUDr. Aleš Linhart CSc¹

¹ Centrum pro plicní hypertenzi, II. interní klinika kardiologie a angiologie, VFN a 1. LF UK, Praha

² II. interní klinika kardiologie a angiologie 1. LF UK a VFN, Praha

Pulmonary hypertension is a heterogenous group of diseases characterized by increase of mean pulmonary arterial pressure. Clinical classification differentiates pulmonary arterial hypertension, pulmonary hypertension in cardiac diseases, pulmonary hypertension in respiratory diseases, chronic thromboembolic pulmonary hypertension and pulmonary hypertension from other causes. The goal of therapeutic intervention is to influence basic pathophysiological mechanisms (vasoconstriction, remodeling and thrombosis). The test of acute pulmonary vasodilatation is essential for decision about the pharmacological treatment. Only patients with a postive test are indicated for treatment with high doses of calcium channel blockers. This therapy with anticoagulation and treatment of heart failure is known as conventional. In case of a negative test besides chronic anticoagulation a specific therapy is indicated (prostanoids, endothelin receptor antagonists, phosphodiesterase 5 inhibitors). In case of exhaustion of pharmacological treatment options, an atrial septostomy and lung transplantation can be considered. Recently it has been shown that similar pharmacological strategies may be effective also in other forms of chronic pulmonary hypertension. In patients with chronic thromboembolic pulmonary hypertension a pulmonary artery endarterectomy is a potentially curable method. In case of exhaustion of all options in advanced cases a lung transplantation is indicated. Because of serious prognosis, sophisticated diagnostic and frequently a complicated therapy it is necessary to concentrate the care of patients with pulmonary hypertension to specialized centers.

Keywords: Key words: pulmonary hypertension, conventional therapy, specific pharmacotherapy, pulmonary artery endarterectomy.

Published: November 1, 2008  Show citation