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Eisenmenger syndrome is a clinical syndrome that develops as a consequence of advanced pulmonary arterial hypertension in a small number of patients with congenital heart disease and left-to- right shunts. It is associated with multi-organ involvement and numerous systemic complications, which greatly affect the quality of life and reduce life expectancy. Diagnosis is made by observing the typical clinical picture of central cyanosis and with the use of different diagnostic methods, most commonly by echocardiography. In patients with Eisenmenger syndrome surgical repair of congenital heart defect is no longer possible. Specific therapy for pulmonary arterial hypertension is available and has been shown to improve quality of life, physical capacity and survival of these patients. In rare cases combined heart and lung or isolated lung transplantation with cardiac defect repair is also possible. The treatment of patients with Eisenmenger syndrome is challenging and should be done in centres specialized in managing adults with congenital heart disease.