NONCARDIAC MORBIDITY IN EISENMENGER SYNDROME – REAL CAUSE OF SEVERE COMPLICATIONS?

Eisenmenger syndrome (ES) represents an extreme form of pulmonary arterial hypertension associated with congenital heart diseases (CHD), but also a severe multisystemic disease. Due to pathologic involvement of several systems, noncardiac morbidity is the most frequent cause of complications or even mortality. Study population. In the group of 47 ES pts (mean age 35 ± 17 yrs) 4 had relevant noncardiac problems (in 2 cholecystopancreatitis requiring cholecystectomy, 1 bleeding from colon, 1 extirpation of benign tumor). For 2 of them managed in regional hospital this complication was lethal. We present a case with management in expert center. Case report. 49yrs old cyanotic female with complex CHD and ES was stable until 3 yrs ago. Due to clinical progression and attacks of hemoptysis specific therapy was initiated, her status improved. Due to progressive growth of tumor glomus caroticus surgical extirpation was indicated and planned at our center in order to prevent complications. Severe secondary erytrocytosis, thrombocytopenia, hemostasis and fibrinolysis activation and capillary fragility were present. Close hematologic management with hemodilution was realized. Hemodynamic monitoring perioperatively was performed, adequate hydratation and analgesia were applied. No complications appeared. Conclusion. Cyanotic pts are due to secondary hematologic and hemocoagulation disturbances threatened by severe bleeding or thrombosis. In ES pts during anesthesia an acute critical pulmonary hypertension, or systemic vasodilatation and hypotension with increased L-R shunting and enhancement of systemic desaturation could appear, so close hemodynamic monitoring is necessary. However these risks can be reduced as in our case by appropriate preoperative preparation and perioperative management, optimally at an experienced GUCH center.