Pulmonary thrombosis in adults with eisenmenger syndrome

The incidence of congenital cardiovascular malformation is unknown but is estimated to be approximately 0.8% of live births. This figure does not include preterm infants (all of whom have a patent ductus arteriosus [PDA]) or stillborn infants (who have a high incidence of cardiovascular abnormalities). Approximately 9% of patients with congenital cardiac disease and 11% of those with uncorrected left-to-right intracardiac shunts will develop the Eisenmenger syndrome (ES). ES develops at different rates, depending on the size and location of the cardiac lesion and the amount of increased pulmonary blood flow and pressure. Onset in infancy is common in patients with PDA or a large ventricular septal defect (VSD). ES is seen in up to 50% of patients with VSD larger than 1.5 cm in diameter, whereas there is only a 3% incidence in those with defects that are less than 1.5 cm in diameter. Children with Down syndrome and a VSD or atrioventricular canal defect are prone to early development of ES. They have abnormal upper airways that predispose them to chronic upper airway obstruction and sleep apnea, both of which promote hypoxemia, hypercarbia, and early development of pulmonary hypertension. ES develops early in nearly all patients with uncorrected transposition of the great arteries and truncus arteriosus, whereas most patients with uncorrected ASD who develop the syndrome do so in the second decade of life. The prognosis for survival through childhood with ES is relatively good. Patients usually lead remarkably active lives until shortly before they die. The goal of this article is to provide a detailed overview of the pathophysiology, clinical presentation, and medical and surgical management of these patients. Particular focus on the anesthetic management of patients undergoing cardiac and noncardiac surgery is provided.