TY - JOUR T1 - Therapeutic approaches in adults with congenital heart disease-associated pulmonary arterial hypertension JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 300 LP - 307 DO - 10.1183/09059180.00004510 VL - 19 IS - 118 AU - R.L. Cordina AU - D.S. Celermajer Y1 - 2010/12/01 UR - http://err.ersjournals.com/content/19/118/300.abstract N2 - Pulmonary arterial hypertension is a major contributor to reduced functional capacity in patients with congenital heart disease. Expert care is essential. Whilst careful supportive management has traditionally been the mainstay for these patients, in recent times significant improvements in exercise capacity and even survival have been observed with the use of disease-targeted therapy, including endothelin receptor antagonists, phosphodiesterase inhibitors and prostanoids. In this review we will discuss current therapeutic options and summarise the recent literature on disease-targeted therapy. ER -