pediatrics
Eisenmenger Syndrome
Introduction
Eisenmenger syndrome is a complication of uncorrected large intracardiac or aortic to pulmonary artery left-to-right shunts. Increased pulmonary resistance may develop over time, eventually leading to severe pulmonary hypertension, bidirectional shunting with progressively increasing right-to-left shunting. Cyanosis and hypoxia inevitably lead to multiple complications discussed below. Physical findings vary depending on the underlying defect and the stage of the pathophysiologic abnormalities. Diagnosis is based on echocardiography or advanced imaging and cardiac catheterization. Once Eisenmenger syndrome occurs, the increased pulmonary vascular resistance and pulmonary hypertension are irreversible, precluding primary correction of the original defect. Therefore, treatment is generally supportive, but heart and lung transplantation may be an option when symptoms are severe. Endocarditis prophylaxis is recommended. (See also Overview of Congenital Cardiovascular Anomalies .) Congenital heart anomalies that, if untreated, may result in Eisenmenger syndrome include (in approximate order of occurrence— General reference ) Ventricular Septal Defect (VSD) (VSD) Atrioventricular Septal Defect (AVSD) Patent Ductus Arteriosus (PDA) (PDA) Aortopulmonary Window None Atrial Septal Defect (ASD) (ASD) Persistent Truncus Arteriosus None Maternal mortality ratios in selected countries (DORV) without pulmonary stenosis Partial anomalous venous return (PAPVR, whereby some of the pulmonary veins connect to the right atrium instead of the left atrium) Rare patients with single ventricle variants or with Transposition of the Great Arteries (TGA) with a persistent patent ductus arteriosus and/or ventricular septal defect may develop Eisenmenger syndrome if untreated, though the vast majority of these patients die early in infancy if the anomaly is not recognized and treated. In the United States, the incidence has markedly decreased because of early diagnosis and definitive repair of the causative anomaly. Right-to-left shunting due to Eisenmenger syndrome results in cyanosis and its complications. Systemic oxygen desaturation leads to clubbing of fingers and toes, Perinatal Polycythemia and Hyperviscosity Syndrome , hyperviscosity, hemoptysis, central nervous system events (eg, brain abscess or stroke), pulmonary artery thrombosis, and sequelae of increased red blood cell turnover (eg, hyperuricemia causing gout, hyperbilirubinemia causing cholelithiasis, iron deficiency with or without anemia). 