Tatiana Valkovičová1, Monika Kaldarárová2, Marcela Boháčeková1, Iveta Šimková1
Eisenmenger syndrome is an epitome of pulmonary arterial hypertension associated with congenital heart disease. Depending on the size and location of the underlying cardiac defect, severe pulmonary arterial hypertension may present with reversed shunting across the defect and associated cyanosis, with all the multisystemic consequences. Though, compared to other types of pulmonary arterial hypertension, patients with Eisenmenger syndrome seem to have better long-term survival. The most important factor for prognosis is considered to be preserved right ventricular function. These patients suffer from chronic hypoxemia, which leads to secondary compensatory erythrocytosis. Together with endothelial dysfunction and haemocoagulation abnormalities it results in multisystemic affection. Authors are presenting the disease development and the most common complications, as well as the management of affected components of the multisystemic disease.