pediatrics
Eisenmenger Syndrome
Key Points
Cardiac anomalies that involve large intracardiac left-to-right shunts often eventually cause increased pulmonary resistance, which first causes bidirectional shunting and ultimately right-to-left shunting (shunt reversal). With shunt reversal, deoxygenated blood enters the systemic circulation, causing hypoxia and its complications (eg, clubbing of fingers and toes, secondary polycythemia); polycythemia may cause hyperviscosity, stroke, or other thromboembolic disorders, and/or hyperuricemia. Symptoms usually do not occur until age 20 to 40 years in patients with pre-tricuspid shunting; in patients with a post-tricuspid shunt, symptoms can occur during the first few years of life. Symptoms include cyanosis, syncope, dyspnea during exertion, fatigue, chest pain, palpitations, atrial and ventricular arrhythmias, hemoptysis, right heart failure, and sudden death. Doing a corrective operation for the underlying cardiac anomaly at the appropriate age should prevent Eisenmenger syndrome. There is no specific treatment once the syndrome develops, other than heart and lung transplantation, but drugs that may lower pulmonary vascular resistance (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) are useful. 