Challenges in managing a critically ill patient with decompensated Eisenmenger syndrome

Abstract

Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension and is associated with congenital heart disease. ES itself is a challenging condition to manage, further compounded if the patient is critically ill and acutely decompensated. We share our experience of managing a critically ill adult patient with ES who presented with acute decompensation due to sepsis.

Venopulmonary ECMO Improved Hypoxemia and Supported the Right Ventricle in a Patient with Decompensated Eisenmenger Syndrome

“…Hypoxemia is typically associated with an increase in hemoglobin concentration, red cell count, and hematocrit (secondary erythrocytosis); it is a compensatory mechanism that increases the oxygen-carrying capacity of the blood by acute extracardiac conditions (infectious processes) or simply by progression secondary to the natural history of the disease that causes an imbalance in the relationship between oxygen supply and consumption and organic damage. When the usual therapy with invasive or noninvasive ventilatory support and the use of vasoactive drugs are not sufficient to restore oxygen supply demand and achieve reversal of tissue hypoxia, ECMO should be considered as a bridge to recovery, a bridge to decision, or a bridge to transplant [13], as in the case of our patient.…”