Perioperative Management of Critical Right Ventricular Inflow Obstruction from Right Atrial Rhabdomyoma

Rhabdomyoma is the most common cardiac tumor in infancy and commonly located in the ventricles causing outflow obstruction or arrhythmias. We report a rare pediatric (7 month old) case of a right atrial rhabdomyoma presenting with severe cyanosis and low cardiac output from significant tricuspid inflow obstruction with right to left shunt across a stretched patent foramen ovale. We present an emergency cardiac surgery for right atrial tumor resection, and the management of separating the patient with failing right ventricle from cardiopulmonary bypass using a Glenn shunt, since extracorporeal membrane oxygenation (ECMO) or nitric oxide was not available.