INTRODUCTION: Pulmonary arterial hypertension (PAH) is a serious primary illness 
of the pulmonary arterioles, characterised by progressive precapillary pulmonary 
hypertension. The conventional therapy for this condition is so-called specific 
pharmacotherapy, which addresses the key mechanisms in the pathophysiology of 
the illness, making use of drugs from the prostanoid group, endothelin receptor 
antagonists and phosphodiesterase inhibitors. Treprostinil is a stable analogue 
of prostacyclin, which can be administered subcutaneously, intravenously or by 
inhalation.
PATIENT SAMPLE AND METHOD: In the centre for pulmonary hypertension in the 
Second Internal Clinic of Cardiology and Angiology of 1st Faculty of Medicine, 
Charles University, and the General Teaching Hospital in Prague, 22 patients 
with PAH (idiopathic PAH, familial PAH, PAH associated with congenital heart 
disease and PAH associated with systemic connective tissue disease) were treated 
with trerpostinil, 18 patients with a continuous subcutaneous infusion and 4 
patients with a continuous intravenous infusion. The indicators followed were 
the distance reached in a 6-minute walking test, functional capacity assessed by 
NYHA classification and mortality.
RESULTS: The patients for whom treprostinil treatment was indicated had an 
average pressure in the right atrium of 11.9 +/- 4.2 mm Hg, average pressure in 
the pulmonary artery of 56.8 +/- 10.7 mm Hg, a cardiac index of 1.78 +/- 0.25 
l/min/m2 and a total pulmonary resistance of 16.26 +/- 4.48 WU. 15 patients were 
functionally NYHA III and 7 patients were NYHA IV. The average distance achieved 
in a 6-minute walk test before the start of treatment was 326 +/- 83 m. When 
treated with gradually increasing doses of treprostinil the distance achieved in 
the 6-minute walk test improved. After 6 months, the group that received 
subcutaneous treatment had extended their distance to 359 m, after 12 months it 
was 393 m, after 24 months 447 m and after 36 months 494 m. After 6 months, the 
group that received intravenous treatment had extended their distance to 473 m, 
which increased to 451 m after 12 months and 489 m after 24 months. Functional 
capacity also improved. In total 5 patients were unable to tolerate the 
subcutaneous infusion, of whom 3 were placed on intravenous treprostinil and 2 
on oral bosentan. 7 of the patients died in the period examined (31.8%).
CONCLUSION: Treprostinil improves symptoms and hemodynamics for PAH patients and 
reduces mortality.