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Aim: To assess correlation between exercise tolerance and Doppler-derived indices of right ventricular (RV)--pulmonary arterial coupling in patients with different location of vascular lesions causing pulmonary hypertension (PH)., Material: 45 patients: 21 with thromboembolic (TE-PH) and 24 with arterial PH defined as systolic pulmonary arterial pressure (SPAP) > 40 mm Hg. The two groups were similar as far as SPAP (74,8 +/- 15,2 vs 76.5 +/- 14,1 mm Hg, p = ns) and distance covered during 6 minute walk test (339 +/- 116 vs 320 +/- 127, p = ns) are concerned., Results: Flow velocity curve in the RV outflow tract were different in the two groups. Time from onset to peak velocity of ejection (acceleration time, AcT) as well as to midsystolic deceleration were shorter in TE-PH then in arterial PH (59.0 +/- 6.3 vs 68.1 +/- 15.4 msec, p = 0.015 and 123.8 +/- 17.9 vs 137.9 +/- 29.7 msec, p = 0.071, respectively). In the whole studied group as well as in arterial PH significant correlation between exercise tolerance and Doppler-derived indices of RV--pulmonary arterial coupling were found (r = 0.51, p < 0.001 and r = 0.72, p = < 0.001, respectively). Interestingly, no such relationship was found in TE-PH, (r = 0.16, p = ns)., Conclusion: In contrast to arterial PH, pressure wave prematurely reflected from the intraluminal thrombi seem to disturb the RV ejection pattern and its correlation with exercise tolerance in TE-PH. Whether progression or effects of treatment on arterial PH can be assessed by monitoring changes in Doppler-derived indices of RV--pulmonary arterial coupling requires further studies.

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.