This review highlights that disease-targeted therapies, such as endothelin receptor antagonists, phosphodiesterase inhibitors, and prostanoids, improve exercise capacity and survival in adults with congenital heart disease-associated pulmonary arterial hypertension.
May support targeted therapies in adult CHD-PAH; leaves open need for RCTs to confirm survival benefit.
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.
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Cordina et al. (2010) studied this question.
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