Plicní arteriální hypertenze (PAH) je potenciálně fatální onemocnění plicních arteriol. Klíčovou roli v patofyziologii onemocnění hraje porucha funkce cévního endotelu charakterizovaná nedostatečnou produkcí vazodilatátorů (NO, prostacyklin) a naopak nadměrnou produkcí vazokonstriktorů (endotelin, tromboxan). V léčbě PAH se kromě konvenční léčby uplatňuje tzv. specifická farmakoterapie (prostanoidy,...

Pulmonary arterial hypertension (PAH) is a potentially fatal disease of pulmonary arterioles. Impaired function of the vascular endothelium characterized by reduced production of vasodilators (NO, prostacyclin) and, conversely, by increased production of vasoconstrictors (endothelin, thromboxane) plays a key role in the pathophysiology of the disease. To treat PAH, apart from conventional therapy, specific pharmacotherapy (prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors) is used, with not only vasodilator but also antiproliferative and antiplatelet effects. Prostanoids (epoprostenol, treprostinil, iloprost and beraprost) have been used for two decades as a primary treatment for PAH. Nevertheless, they are not easy to use and are associated with a range of complications. The action of prostanoids is mediated by binding to membrane receptors and then intracellularly by binding to transcription factors from the class of peroxisome proliferator-activated receptors (PPARs). Epoprostenol has a very short half-life and that is why it needs to be given as continuous intravenous infusion. It is the most effective treatment in the most advanced stages of PAH. Treprostinil, as a more stable drug, is administered as continuous subcutaneous infusion, can be given intravenously or used as an inhaled and possibly also oral medication. Iloprost is prescribed as an inhaled treatment in PAH. Beraprost is the only authorized oral prostacyclin analogue. Nevertheless, it has proved effective in less severe forms of PAH only.

Centrum pro plicní hypertenzi 2 interní klinika kardiologie a angiologie VFN a 1 LF UK Praha

Prostanoids in the treatment of pulmonary arterial hypertension

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