RT Journal Article
SR Electronic
T1 Double combination therapy in patients with pulmonary arterial hypertension associated with congenital heart defects
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p2284
VO 38
IS Suppl 55
A1 Nicole Rizzo
A1 Elisa Conficoni
A1 Massimiliano Palazzini
A1 Enri Leci
A1 Enrico Gotti
A1 Gaia Mazzanti
A1 Francesca Terzi
A1 Alessandra Manes
A1 Elena Beciani
A1 Francesca Sciarra
A1 Cristina Bachetti
A1 Federica Sgro'
A1 Nazzareno Galiè
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p2284.abstract
AB Purpose: Patients with pulmonary arterial hypertension associated with congenital heart defects (PAH-CHD) are currently treated with targeted therapy. According to recent guidelines PAH-CHD patients with inadequate clinical response should also be treated with combination therapy (CT). We assessed the effects of double CT in a group of PAH-CHD patients.Methods: In the last 6 years, 43 adult patients with PAH-CHD (age 43±14 ys, 65% females) already treated with monotherapy were included. Twenty-nine were treated with bosentan (125 mg bid) and 14 were treated with sidenafil (20 or 25 mg tid). Twelve patients had a ventricular septal defect, 5 patients had an atrial septal defect, 5 had a patent ductus arteriosus, 8 had combined defects, and 13 had corrected defects. At baseline and after CT (with sildenafil or bosentan according with the first-line treatment) 6-minute walk test (6MWT) and right-heart catheterization were performed.Results: One patient did not perform 6MWT and another one did not undergo RHC after combination therapy. The table shows the haemodynamic and functional changes after a mean treatment period of 4.8±2.1 months of CT.View this table:Conclusions: CT improves exercise capacity and hemodynamics in patients with PAH-CHD already on monotherapy. Sequential CT appears to be an appropriate approach also in patients with PAH-CHD.