pediatrics
Eisenmenger Syndrome
Diagnosis of Eisenmenger Syndrome
Chest x-ray and ECG Echocardiography or cardiac catheterization Diagnosis of Eisenmenger syndrome is suspected by history of uncorrected cardiac anomalies, supported by chest x-ray and ECG findings, and established by 2-dimensional echocardiography with color flow and Doppler studies. Cardiac catheterization is often done to measure pulmonary artery pressure, pulmonary vascular resistance, and response to pulmonary vasodilators. Laboratory testing shows polycythemia with hematocrit>55%. Increased red blood cell turnover may be reflected as an iron deficiency state (eg, microcythemia), hyperuricemia, and hyperbilirubinemia. Iron deficiency should be suspected if the mean corpuscular volume (MCV) is low; iron deficiency can be identified by measuring transferrin saturation and ferritin. Chest x-ray usually shows prominent central pulmonary arteries, peripheral pulmonary vessel pruning, and right heart enlargement. ECG shows right ventricular hypertrophy, right axis deviation, and, occasionally, right atrial enlargement. 