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Infant Cardiac Rhabdomyoma With Incessant Arrhythmia: Role of Tumor Debulking and Margin Cryoablation
John P Costello1, Nicholas Oh1, Miza Salim Hammoud1
1Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic Children's Hospital and Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio.
Insights
Cardiac rhabdomyomas can cause persistent atrial arrhythmias in infants. Surgical debulking and cryoablation of residual tumor effectively resolved arrhythmias, offering a potential treatment pathway.
Area of Science:
- Pediatric Cardiology
- Cardiac Surgery
- Oncology
Background:
- Cardiac rhabdomyomas are benign tumors associated with tuberous sclerosis.
- Atrial arrhythmias can complicate rhabdomyoma management, leading to significant morbidity.
- Current treatments often focus on medical management, with limited success in refractory cases.
Observation:
- A 4-month-old infant presented with drug-refractory atrial arrhythmias secondary to cardiac rhabdomyomas.
- Despite maximal medical therapy and extracorporeal membrane oxygenation, arrhythmias persisted.
- Surgical intervention was required to address the underlying tumor burden.
Findings:
- Surgical debulking of the atrial rhabdomyoma was performed under cardiopulmonary bypass.
- A residual tumor rim on the right atrial free wall was treated with cryoablation.
- The patient was successfully weaned off antiarrhythmic medications and extracorporeal support.
Implications:
- This case demonstrates the feasibility and efficacy of surgical tumor resection combined with cryoablation for managing refractory atrial arrhythmias in infants with cardiac rhabdomyomas.
- Targeted surgical intervention offers a potential curative approach for this challenging clinical scenario.
- Long-term follow-up confirmed sustained arrhythmia freedom, highlighting the durable benefits of this combined therapeutic strategy.
Abstract:
We describe the management of a 4-month-old girl (5.0 kg) who presented with atrial arrhythmias in the setting of cardiac rhabdomyomas. Despite maximal medical therapy and extracorporeal membrane oxygenation support, atrial arrhythmias persisted. Surgical tumor debulking of the atrial mass was undertaken on bypass, and a rim of residual tumor on the right atrial free wall was cryoablated. The patient was weaned off antiarrhythmics and discharged on postoperative day 15 and has been arrhythmia free in 14 months of follow-up. Rhabdomyoma resolution is possible with targeted resection and cryoablation of any remaining tumor at the resection margin.

