Therapeutic approaches in adults with congenital heart disease-associated pulmonary arterial hypertension

被引:7
作者
Cordina, R. L. [1 ]
Celermajer, D. S.
机构
[1] Univ Sydney, Royal Prince Alfred Hosp, Sydney, NSW, Australia
关键词
Atrial septal defect; congenital heart disease; Eisenmenger syndrome; left-to-right shunt; pulmonary arterial hypertension; ventricular septal defect;
D O I
10.1183/09059180.00004510
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
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.
引用
收藏
页码:300 / 307
页数:8
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