Abstract
Objective: Evaluate the data of right heart catheterization (RHC) and echocardiography (ECHO) at baseline and their dynamics during 12-week treatment with bosentan in patients with idiopathic pulmonary hypertension (IPH). Design and method: The study included 42 patients (39 women and 4 men, II-IV FC (WHO)) with a verified diagnosis IPH. All patients underwent acute pharmacological testing (aPhT) with iNO during RHC. Patients with negative vasoreactivity response (n = 22) recieved pathogenetic therapy with bosentan and standard therapy of heart failure. The starting dose was 62,5 mg twice per day for 1 month, followed by titration to dose 125 mg twice/day. Dose titration was carried out under the control of blood pressure, heart rate, liver enzymes monthly. Evaluating the effectiveness of treatment was carried out according to RHC and ECHO. Results: The therapy increased exercise tolerance according to 6MWT: initially 363,2 ± 80,4m. and during 3 months of therapy 427,8 ± 69,9 m. (p < 0,05). There was improvement of hemodynamic parameters according to RHC: decreased mean pulmonary artery pressure of 8,8mmHg (p < 0,05), pulmonary vascular resistance of 602,6din*s/sm5 (p < 0,05); cardiac index increased by 0,6 l /min*m2 (p < 0,05) at the same time there have been no significant reduction in systolic pulmonary artery pressure (initially 102,4 mmHg, on the treatment 96,4 mmHg (p > 0,05)). According to echocardiography there was a significant positive dynamic: reduced antero-posterior size of the right ventricle (delta = -0,23sm), area of the right atrium (delta = -1,6sm2); increased end-diastolic size of left ventricule (delta = +0,3sm). Conclusions: The patients had positive dynamics in the form of increased exercise tolerance, improvement data of RHC and echocardiography during 12 weeks of bosentan therapy.
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