Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option.
A 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 ( Tracleer ) or Sildenafil ( Revatio ) 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 less than 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. ( Xagena )
Dardi F et al, Eur Respir J 2015;46:414-421