Current therapy of Eisenmenger syndrome

Eisenmenger syndrome (ES) is a complex and disastrous medical problem with profound cyanosis and clinical deterioration by significant right to left shunting. This syndrome is the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD). Inverted shunt changes structures of pulmonary vasculature like all forms of PAH in ES (1). In the past, the management of patients with ES was limited to conventional therapy with an emphasis on regular informed cardiovascular follow-up. Subsequent clinical studies have made it possible to improve to patient survival and functional capacity. There are major therapeutic targets in PAH treatment using endothelin-receptor antagonists, phosphodiesterase type-5 inhibitors, and prostacyclin derivatives.