Aim of study: assess the efficacy of treatment with phosphodiesterase inhibitors type 5 monotherapy in children with PAH and congenital heart disease. Methods: 7 patients with PAH and CHD who received phosphodiesterase inhibitors type 5 were included in this study. The treatment response was assessed by: exercise tolerance in 6MWT, NYHA functional class change, echocardiographic monitoring. Results: significant decrease of systolic pulmonary arterial pressure (from 112.14±22.14 to 85.71±34.08 mmHg, p=0.0277), TRPG (from 100±22.17 to 75.42±34.6, p=0.0277) and PVR (from 7.22±2.04 to 4.06±1.23, p=0.0431) and increase of TVIT (from 352.83±99.14 to 429.83±103.94, p=0.0277) and PAAcT (from 76.57±24.17 to 89±16.63, p=0.0431).. 6MWT increased a mean of 73m. Conclusions: phosphodiesterase inhibitors type 5 were well tolerated and improve both pulmonary haemodynamics and the clinical status after 3 months therapy.
Keywords: pulmonary hypertension, congenital heart disease