RT Journal Article
SR Electronic
T1 Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 414
OP 421
DO 10.1183/09031936.00209914
VO 46
IS 2
A1 Dardi, Fabio
A1 Manes, Alessandra
A1 Palazzini, Massimiliano
A1 Bachetti, Cristina
A1 Mazzanti, Gaia
A1 Rinaldi, Andrea
A1 Albini, Alessandra
A1 Gotti, Enrico
A1 Monti, Enrico
A1 Bacchi Reggiani, Maria Letizia
A1 Galiè, Nazzareno
YR 2015
UL http://erj.ersjournals.com/content/46/2/414.abstract
AB Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option.This study aimed to assess the impact of sequential combination therapy on both short-term responses and long-term outcomes in a real-world setting.Patients with idiopathic/heritable pulmonary arterial hypertension, or pulmonary arterial hypertension associated with congenital heart disease or connective tissue disease and who were not meeting treatment goals on either first-line bosentan or sildenafil monotherapy, were given additional sildenafil or bosentan and assessed after 3–4 months. Double combination therapy significantly improved clinical and haemodynamic parameters, independent of aetiology or the order of drug administration. Significant improvements in functional class were observed in patients with idiopathic/heritable pulmonary arterial hypertension. The 1-, 3- and 5-year overall survival estimates were 91%, 69% and 59%, respectively. Patients with pulmonary arterial hypertension associated with connective tissue disease had significantly poorer survival rates compared to other aetiologies (p<0.003).The favourable short-term haemodynamic results and good survival rates, observed in patients receiving both bosentan and sildenafil, supports the use of sequential combination therapy in patients failing on monotherapy in a real-world setting.Bosentan and sildenafil combination therapy improved haemodynamics and exercise in PAH patients failing monotherapy http://ow.ly/LGrPm