Eisenmenger syndrome--a unique form of pulmonary arterial hypertension.

In 5-10% of adults with congenital heart disease (left-to-right shunt defects), pulmonary arterial hypertension (PAH) can develop with variable severity. An extreme manifestation of PAH in this setting is known as the Eisenmenger syndrome. It represents not only PAH associated with congenital heart disease, but also a multi-systemic disorder, presented by variety of complications (cyanosis, bleeding, thrombotic diathesis, high risk of bacterial endocarditis or cerebral abscess, ischemic complications, hepatic and renal involvement, congestive heart failure and sudden death). Authors concisely identify the underlying pathophysiological and hemodynamic aspects of Eisenmenger syndrome and focus on the clinical presentation. Eisenmenger syndrome represents a unique form of PAH with many differences. The preserved right ventricular function seems to play the key role in a better survival of these patients compared to other forms of PAH. To achieve a satisfactory prognosis and life-quality in patients with Eisenmenger syndrome it is necessary not only to treat their hemodynamic features (PAH and/or ventricular dysfunction) but also to adequately manage and prevent all the multi-systemic complications resulting from this disease. This often needs a care in specialized centers with multi-disciplinary approach (Tab. 2, Fig. 6, Ref. 17).