Eisenmenger's Syndrome

Key points

• Reversal of a left-to-right shunt in a congenital heart defect due to pulmonary hypertension
• Mechanism: uncorrected left-to-right shunt → remodelling of the pulmonary microvasculature → obstruction to pulmonary blood flow → pulmonary hypertension
• Associated with: ventricular septal defect · atrial septal defect · patent ductus arteriosus
• Features: the original murmur may disappear · cyanosis · clubbing · right ventricular failure · haemoptysis, embolism
• Management: heart-lung transplantation is required