Clinical and hemodynamic picture of Eisenmenger syndrome.

Eisenmenger syndrome represents a very specific form of pulmonary arterial hypertension (PAH). Unlike patients with idiopathic PAH, in Eisenmenger syndrome the clinical and cardiac status is often relatively stable for a long time. On the other hand, due to cyanosis and due to maladaptive body reactions many noncardiac complications may occur. Fourteen patients (pts) with Eisenmenger syndrome were analyzed, with the mean age 41 years. Invasively measured pre-capillary pulmonary hypertension was severe (mean pulmonary arterial pressure 79 mmHg) and a statistically significant difference according to the site of defect was found (pre-tricuspid shunts vs post-tricuspid shunts = 57,5 mmHg vs 88 mmHg; p = 0.01). It is neccessary to keep in mind that non-cardiac events and complications may lead to death sooner then the right ventricular dysfunction or PAH (Tab. 7, Fig. 21, Ref. 16).