Therapeutic approaches in adults with congenital heart disease-associated pulmonary arterial hypertension.
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.
University of Sydney, Sydney Australia.
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Cordina
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Celermajer
D065128:Endothelin Receptor Antagonists; D006330:Heart Defects, Congenital / Q000150:complications* / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000401:mortality / Q000628:therapy*; D010100:Oxygen / Q000627:therapeutic use; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011453:Prostaglandins / Q000627:therapeutic use; D015996:Survival Rate; D016896:Treatment Outcome
D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins; D010100:Oxygen