Clinical and hemodynamic profile and neurohormonal indicators in patients with idiopathic pulmonary hypertension, depending on vasoreactivity reserve of pulmonary arteries

Clinical and hemodynamic profile and neurohormonal indicators in patients with idiopathic pulmonary hypertension, depending on vasoreactivity reserve of pulmonary arteries. Objective: the main objective of our research work was an evaluation of clinical and hemodynamic profile, neurohormonal indicators in patients with IPH, depending on the results of vasoreactivity testing. Methods: the study included 131 patients with IPH (mean age 32,4±3,2 years. All the patients underwent the routine laboratory tests, 6-minuts walking test (6-MWT), transthoracic echocardiography, thorax organs radiography, right heart catheterization (RHC) with acute pharmacological test (APT) using one or two vasodilators (VD) In order to study neurohumoral status the analysis of the 6-keto-PgF1β, NO, ET-1, norepinephrine, epinephrine levels, NT-proANP, NT-proBNP (NUP), renin activity, AII, aldosterone (RAAS) activity was conducted. Results: significant differences of clinical and hemodynamic profile and neurohormonal indicators in patients with IPH depending on the availability (APT +) and absence (APT-) of the vasoreactivity reserve of pulmonary arteries (PA) were established. Among the APT- patients the number of patients belonging to FC III was significantly greater in comparison with APT+ patients. FC IV refered only to the APT- patients. Significantly more patients with APT+ belonged to FC I and II. The 6-MWT revealed that patients with IPH with intact vasodilation reserve overcame significantly longer distance. Doppler echocardiography showed that in APT-group a greater level of systolic pressure in the pulmonary artery (SPPA). When comparing the RHDC data all the hemodynamic parameters in the groups varied considerably, prognostically better data marked APT+ group. According to thorax organs radiography APT- group had significantly higher leel of KTI. Comparative evaluation of neurohumoral status, regardless of the outcome of the APT, showed a significant difference in the groups. Conclusion: the group of responders in comparison with the APT- patients group indicated more safe clinical-hemodynamic and functional status, and neurohumoral rates in the responders group were closer to the outcome in the control group.