The first Russian experience of sildenafil application in patients with idiopathic pulmonary hypertension

Abstract

Over the last decade, the interest in the treatment of idiopathic pulmonary hypertension (IPAH) has greatly increased because of the development and implementation of innovative therapeutic approaches. One of the modern drug groups for the treatment of pulmonary arterial hypertension are phosphodiesterase type 5 inhibitors (PDE5), which can cause pulmonary vasodilation via a NO-dependent mechanism.The aim of the research was to study the efficacy and safety of sildenafil treatment in patients with IPAH received 16-week course and to study sildenafil impact on clinical, hemodynamic and functional status.Material and methods: 20 patients with IPAH (mean age 40.9±11.5 years) functional class (FC) II-IV (WHO), who were receiving standard therapy (anticoagulants, diuretics, glycosides, calcium antagonists), were prescribed sildenafil 20 mg three times a day for 16 weeks. Initially, and after 16 weeks of treatment we studied FC, and the results of the six-minute walk test (6MWT), transthoracic echocardiography (ECHO), right heart catheterization (RHC) and laboratory tests to determine the safety of the therapy were carried out, as well.Results: the group of patients had 6MWT distance of 344±116 m at the beginning of the study. For about 90% of the patients suffered from dizziness, 75% - from the pain in the heart, 65% of the patients had edema and 35% - hepatomegaly. The patients suffered from severe pulmonary hypertension according to the functional and hemodynamic status. The estimated pulmonary artery systolic pressure (PASP) according to ECHO results was 89.6±22 mm Hg. According to the data from RHC we noted the elevation of mean pulmonary arterial pressure (mPAP) to 56.3±19 mm Hg, of pulmonary vascular resistance (PVR) to 1218±709 dyne/sec x per x cm-5 and the decrease in cardiac output (CO) - 3.4±0.8 l/min/m2.There were slight increasing in 6MWT distance (+23 m, p>0.05) and a statistically significant improvement in clinical status in comparison with the initial data: dizziness - 71%, heart pain - 38%, edema and hepatomegaly were observed in 15% and 14% (p