Abstract
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.
- Atrial septal defect
- congenital heart disease
- Eisenmenger syndrome
- left-to-right shunt
- pulmonary arterial hypertension
- ventricular septal defect
Footnotes
Provenance
Publication of this peer-reviewed article was supported by Actelion Pharmaceuticals Ltd, Switzerland (unrestricted grant, European Respiratory Review issue 118).
Statement of Interest
D.S. Celermajer has attended PAH seminars over the last 5 yrs and has been sponsored to attend some of these events by Actelion, who make medications for the treatment of pulmonary arterial hypertension. He has also received speaker fees and received research support from Actelion for studies in pulmonary arterial hypertension.
- Received April 21, 2010.
- Accepted May 3, 2010.
- ©ERS 2010