CLINICAL AND HEMODYNAMIC PROFILE OF PATIENTS WITH IDIOPATHIC ARTERIAL PULMONARY HYPERTENSION DEPENDING ON PULMONARY ARTERY VASODILATOR REACTIVITY

Objective: To comparethe clinical and hemodynamic status of idiopathic arterial pulmonary hypertension (IPAH) patients withpositive and negative results of acute vasodilator testing with nitric oxide (iNO). Design and method: Inthestudyweincluded 131 ptswithIPAH(mean age 32,4 ± 3,2 years).All pts were underwent clinical examination, Echo, 6-min walking test, routine lab tests, acute testing (AT) with iNO 20 ppm at RHC with the guidelines criteria of positive AT. According to acutevasodilator testing the pts were divided into two groups depending on iNO response(n = 51) or nonresponse(n = 80). Results: % of iNO responders among IPAH group was 31%. The IPAH responder group (AT+) was characterized by highest% of pts with functional class (FC) II (71,4%) with the lowest proportion of patients with FC III (17,9%). In T6MWIPAH responders had significantly greater distance: 433,94 ± 77,89 as compared to AT- group(320,04 ± 108,19m), which was accompanied by a less severe dyspnea index (2,59 ± 1,46 vs. 4,25 ± 1,86). AT- group was characterized by significantly greater systolic presser artery pulmonary(SPAP)increase, more pronounced dilatation and hypertrophy of RV, the smaller size of LV.At right heart catheterization we found significant differences of almost all measured and calculated parametersbetween the groups with AT- and AT +:SPAP- 101,41 ± 24,88 vs 80,14 ± 15,53 mmHg(p < 0,000001), PAPm- 66,2 ± 17,99 vs 51,22 ± 9,05mmHg (p = 0,0000008), SV- 3,11 ± 0,86 vs 4,13 ± 1,05 l /min (p < 0,0000005), PVR- 1439,94 ± 667,16 vs 935,51 ± 479,55 dinxsxcm5(p = 0,000008). Conclusions: At the time of initial diagnosis ofIPAH the pts with preserved pulmonary artery vasodilator reactivity to iNO were characterized by favorable functional/hemodynamic profile and less pronounced heart remodeling.