Anesthetic Care During Posterior Spinal Fusion in an Adolescent With Ebstein's Anomaly

Ebstein's anomaly is a rare form of cyanotic congenital heart disease (CHD) that involves malformation and dysfunction of the tricuspid valve and right ventricle (RV). The severity of the defect impacts clinical presentation, survival, and treatment options. Presentation during the neonatal period with hypoxemia and cyanosis is noted in patients with severe tricuspid valve malformation, a hypoplastic RV, or RV outflow tract obstruction. However, presentation later in infancy is more common when there is a moderate tricuspid valve malformation and no associated RV outflow tract obstruction. Although Ebstein's anomaly is not generally associated with other congenital defects, patients may occasionally require surgery for other comorbid conditions. We describe the perioperative anesthetic management of an adolescent with Ebstein's anomaly for posterior spinal fusion. Previous reports of anesthetic care in this clinical scenario are reviewed, anesthetic considerations discussed, and options for intraoperative monitoring and anesthetic care presented.