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Management of Adults With Congenital Heart Disease

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85 2022 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension 2025 ACC/AHA Guidelines for the Management of Adults with Congenital Heart Disease Bosentan is a class 1 recommendation; other endothelin receptor antagonists "should be considered" in patients with Eisenmenger syndrome (class 2a recommendations). All endothelin receptor antagonists are recommended as initial treatment in patients with Eisenmenger syndrome (class 1 recommendation). Phosphodiesterase-5 inhibitors "should be considered" in patients with Eisenmenger syndrome (class 2a recommendation). All phosphodiesterase-5 inhibitors are recommended as initial treatment in patients with Eisenmenger syndrome (class 1 recommendation). Riociguat "should be considered" in patients with Eisenmenger syndrome (class 2a recommendation). No specific recommendation due to lack of data. Initial prostacyclin therapy "should be considered" in patients with Eisenmenger syndrome (class 2a recommendation). Initial inhaled prostacyclin therapy may be considered in patients with Eisenmenger syndrome (class 2b recommendation). No recommendation No recommendation

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