Open AccessTherapeutic approaches in adults with congenital heart disease-associated pulmonary arterial hypertension
Author(s) -
Rachael Cordina,
D. Celermajer
Publication year - 2010
Publication title -
european respiratory review
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.565
H-Index - 66
eISSN - 1600-0617
pISSN - 0905-9180
DOI - 10.1183/09059180.00004510
Subject(s) - medicine , intensive care medicine , pulmonary hypertension , disease , heart disease , endothelin receptor , cardiology , receptor
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.