Key result
Right atrial tumor resection and Glenn shunt manages critical RV inflow obstruction in an infant.
Why the study?
Right atrial rhabdomyoma causing critical tricuspid inflow obstruction with right to left shunt is a rare and severe condition requiring specific perioperative management strategies.
Case Report (n=1)
A Glenn shunt can be used as a rescue strategy to separate a patient with a failing right ventricle from cardiopulmonary bypass after right atrial tumor resection when ECMO or nitric oxide are unavailable.
May offer rescue in rare infant RV obstruction without ECMO/NO; leaves open need for validation before wider adoption.
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.
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Norawat et al. (2018) conducted a case report in Right atrial rhabdomyoma with critical right ventricular inflow obstruction (n=1). Emergency cardiac surgery for right atrial tumor resection and Glenn shunt was evaluated. Emergency cardiac surgery with right atrial tumor resection and a Glenn shunt was utilized to manage a 7-month-old infant with critical right ventricular inflow obstruction.
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