Over the last decade advanced therapies for the management of pulmonary arterial hypertension have been
introduced. These agents have also been effective in reducing pulmonary vascular resistance in patients with Eisenmenger
syndrome. Specific guidelines focusing on modern therapies for Eisenmenger syndrome however do not exist to date.
More recently, clinical trials in patients with Eisenmenger syndrome demonstrated a significant clinical improvement with
favorable safety and tolerability profile. This review aims to summarize newly reported pharmacological agents used in
patients with Eisenmenger syndrome.
Keywords: Congenital heart disease, endothelin receptor antagonists, eisenmenger syndrome, phosphodiesterase type-5
inhibitors, prostanoids, pulmonary arterial hypertension.
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