Double Outlet Right Ventricle

Abstract

Anesthetizing a patient with double outlet right ventricle (DORV) requires knowledge of a wide spectrum of anatomic and physiologic variants. Caring for these patients includes everything from preparing for a patient-limited pulmonary blood flow due to either fixed or dynamic right ventricular outflow obstruction similar to tetralogy of Fallot (TOF), or preparing for one with unrestricted pulmonary overcirculation. With an incidence of 3–9/100,000 live births, this lesion is relatively rare. But the physiologic considerations of managing these patients are important to those who care for patients with congenital heart disease, both for when they encounter these patients, and also because the principles guiding the management of these patients are applicable to many others, such as those with transposition of the great arteries or TOF. Here we review the anatomic and physiologic considerations of managing these patients, and frame the chapter with clinical vignettes to highlight anesthetic management goals.