THE IMPORTANCE OF ENDOTHELIAL DYSFUNCTION FOR THE DEVELOPMENT OF PULMONARY ARTERIAL HYPERTENSION AND NEW THERAPEUTIC OPTIONS

Abstract

Background. Pulmonary arterial hypertension may exist as a primary condition or as a secondary condition, most commonly with collagen vascular disease. Patients with pulmonary arterial hypertension usually have bad prognosis as the effectiveness of conventional treatment is poor. New knowledge about importance of endothelial dysfunction in development of pulmonary arterial hypertension has opened a wide field of possible new treatment options.Conclusions. Endothelial dysfunction in pulmonary vascular bed is characterised by an inappropriate release of vasoactive substances from endothelium producing vasoconstriction, remodeling of arterial wall and increased local tendency for thrombosis. Several new drugs have been already tested in clinical case-controlled studies: prostacyclin and its analogues applied intravenously or in inhalations, inhaled nitric oxide and drugs which increase nitric oxide concentration such as sildenafil and l-arginine, endothelin receptors antagonists in tablets and tromboxane antagonist in tablets. Only tromboxane antagonist was shown to be ineffective whereas all other drugs were effective at least in certain subgroups of patients. New clinical trials will give answer not only in respect to the comparative efficacy of the drugs mentioned earlier and their combinations but also to the recognition of factors that determine the success of treatment in individual patient.

Keywords

• pulmonary arterial hypertension
• endothelial dysfunction
• treatment