title: [Effect of long-term therapy with oral Beraprost on survival of patients with arterial and inoperable thromboembolic pulmonary hypertension].
abstract: Beraprost sodium (BPS)--an orally active prostacyclin analogue--improves haemodynamic parameters and quality of life in group of patients with pulmonary arterial hypertension. Effect of long-term therapy with BPS is not well defined. This study assesses influence of long-term therapy with BPS on the survival of patients with precapillary pulmonary hypertension. Studied group consisted of 25 patients with precapillary PH (18 F, 7M, aged 34 +/- 13,9 years). Sixteen patients were diagnosed with primary PH, 3 pts had PH associated with connective tissue disease, 5 pts developed PH in course of congenital systemic to pulmonary shunt, and 1 patient suffered from inoperable chronic thromboembolic PH. At time of diagnosis 15 pts presented exercise impairment of WHO class II and 10 pts were in functional class III. All studied subjects had complete hemodynamic assessment of right heart and obtained values were used for estimation of hypothetic survival using prognostic equation proposed by D'Alonzo et al. On follow-up period patients received BPS in the highest tolerated dose (80-480 mg daily). During a follow-up period (mean: 22 months) 7 patients died. Cumulative survival rate BPS group was significantly higher in BPS group comparing to hypothetical survival at 6 months (96% (95% CI: 88-104%) vs 73% (95% CI: 67-78%), p = 0.02) and 12 months (94% (95% CI: 84-104%) vs 65% (58-71%), p = 0.01), respectively. At 18 and 24 months differences between BPS virtual and hypothetical survival were not statistically significant. There was no correlation between survival and maximal achieved dose of BPS. These results suggest, that BPS improves prognosis of patients with precapillary PH during 12 months after initiation of therapy. Later effect of BPS seems to decrease, requiring changing or intensification of therapy.
authors.affiliation: Klinika Chorób Wewnetrznych, Instytutu Gruźlicy i Chorób Płuc w Warszawie. mkurzyna@amwaw.edu.pl
authors.forename: Marcin
authors.initials: M
authors.lastname: Kurzyna
authors.forename: Michał
authors.initials: M
authors.lastname: Florczyk
authors.forename: Anna
authors.initials: A
authors.lastname: Fijałkowska
authors.forename: Paweł
authors.initials: P
authors.lastname: Kuca
authors.forename: Grzegorz
authors.initials: G
authors.lastname: Szewczyk
authors.forename: Janusz
authors.initials: J
authors.lastname: Burakowski
authors.forename: Jarosław
authors.initials: J
authors.lastname: Kober
authors.forename: Jarosław
authors.initials: J
authors.lastname: Sikora
authors.forename: Liliana
authors.initials: L
authors.lastname: Wawrzyńska
authors.forename: Monika
authors.initials: M
authors.lastname: Szturmowicz
authors.forename: Witold
authors.initials: W
authors.lastname: Tomkowskil
authors.forename: Adam
authors.initials: A
authors.lastname: Torbicki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D004334:Drug Administration Schedule; D011464:Epoprostenol / Q000008:administration & dosage* / Q000031:analogs & derivatives*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage*; D016019:Survival Analysis; D013923:Thromboembolism / Q000150:complications*; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D010975:Platelet Aggregation Inhibitors; D014665:Vasodilator Agents; C048081:beraprost; D011464:Epoprostenol