[Two-year follow-up of pulmonary arterial hypertension patients treated with sildenafil].

Background: The long-term effects of drugs developed for the control of pulmonary arterial hypertension (PAH) are little known, since multicenter studies usually last 12 to 16 weeks.

Objective: To evaluate the two-year outcome of PAH patients receiving monotherapy with sildenafil (a phosphodiesterase-5 inhibitor), with regard to their functional capacity.

Methods: Twenty four patients (ages between 8 and 54 years) with idiopathic PAH (IPAH, n = 9) or congenital heart disease-associated PAH (CHD-PAH, n = 15) were treated with sildenafil for two years, with daily oral doses ranging from 60 to 225 mg (tid).

[Doppler echocardiography and hemodynamic parameters in congenital heart disease with increased pulmonary flow].

Background: The prediction of pulmonary hemodynamic data from non-invasive assessment could exempt some patients with congenital cardiac septal defects from preoperative invasive assessment (catheterization).

Objective: To determine, in simultaneous assessment, whether data obtained from Doppler echocardiography could predict aspects of pulmonary hemodynamics in such patients.

Methods: Echocardiographic parameters related to systolic and systemic pulmonary flow and pulmonary venous flow were related to hemodynamic data in 30 consecutive patients with cardiac septal defects (aged 4 months to 58 years, median 2.