Perioperative management of critical right ventricular inflow obstruction from right atrial rhabdomyoma

Area of Science:

• Pediatric Cardiology
• Cardiac Surgery
• Oncology

Background:

• Rhabdomyoma is the most common cardiac tumor in infants, typically found in ventricles.
• These tumors can cause significant hemodynamic compromise, including outflow obstruction and arrhythmias.

Observation:

• A 7-month-old infant presented with severe cyanosis and low cardiac output.
• The cause was a rare right atrial rhabdomyoma obstructing tricuspid inflow and causing a right-to-left shunt via a patent foramen ovale.

Findings:

• Emergency cardiac surgery successfully resected the right atrial rhabdomyoma.
• A Glenn shunt was employed to manage right ventricular failure and facilitate separation from cardiopulmonary bypass, as ECMO and nitric oxide were unavailable.

Implications:

• This case highlights the importance of considering rare cardiac tumors in pediatric patients with cyanosis and low cardiac output.
• The successful use of a Glenn shunt in this emergent scenario demonstrates its utility when advanced support like ECMO is unavailable.
• Early diagnosis and surgical intervention are critical for managing symptomatic cardiac rhabdomyomas in infants.