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Surgical debulking of large ventricular fibromas in children
Ajami Gikandi1, Peter Chiu1, Jordan Secor1
1Division of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Mass.
Insights
Surgical resection of large ventricular fibromas in children is safe, with debulking improving outcomes. Most children maintain heart function and remain arrhythmia-free post-surgery.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Pediatric Oncology
Background:
- Ventricular fibromas are rare pediatric cardiac tumors.
- Surgical management presents unique challenges due to tumor location and potential for obstruction.
Purpose of the Study:
- To update on the clinical presentation, diagnostic workup, operative strategies, and midterm outcomes of ventricular fibroma resection in children.
- To evaluate the safety and efficacy of surgical debulking for pediatric ventricular fibromas.
Main Methods:
- Retrospective cohort study of 52 children undergoing ventricular fibroma resection (2000-2023).
- Analysis of clinical data, surgical techniques (including debulking), and midterm follow-up outcomes.
Main Results:
- Surgery indicated for arrhythmia (86%), symptoms (27%), or hemodynamic compromise (21%).
- Tumor distorted AV valve in 58% and abutted coronary arteries in 79%.
- No mortality or heart transplants; 15-year reoperation risk 6.7%, ventricular tachycardia/fibrillation risk 2.4%. Left ventricular ejection fraction remained stable post-debulking.
Conclusions:
- Ventricular fibroma resection is safe with careful surgical planning and debulking.
- Children maintain left ventricular function and remain free of recurrent arrhythmias at midterm follow-up.
- Long-term surveillance is necessary to assess risks of scar-related arrhythmias.
Objective:
This study aims to provide an update on the clinical presentation, diagnostic workup, operative strategies, and midterm outcomes in children undergoing ventricular fibroma resection.
Methods:
Single-center, retrospective cohort study of patients undergoing ventricular fibroma resection between 2000 and 2023.
Results:
Among 52 patients, median age at surgery was 2.0 years (interquartile range, 0.8-4.6) and median tumor volume index was 69 mL/m2 (interquartile range, 49-169). Tumor distorted the atrioventricular valve/subvalvar apparatus in 30 patients (58%) and abutted major epicardial coronary arteries in 41 patients (79%). Surgery was indicated for arrythmia (n = 45, 86%), symptoms (n = 14, 27%), or hemodynamic compromise (n = 11, 21%). Tumor was debulked in 34 patients (65%), including the last 21 patients. Concomitant atrioventricular valvuloplasty was performed in 18 patients and ventricular cavity closure in 15 patients (29%). During a median follow-up of 2.4 years (interquartile range, 0.8-6.2), there was no mortality, cardiac arrests, heart transplants, or single ventricle palliation. The 15-year risk of reoperation and clinical ventricular tachycardia/fibrillation was 6.7% (95% CI, 0-14.3) and 2.4% (95% CI, 0-7.2), respectively. On latest imaging, pre- and postdebulking left ventricular ejection fraction did not significantly differ (P = .069), whereas no patients had signs of outflow tract obstruction, inflow tract obstruction, or moderate or greater atrioventricular valve regurgitation.
Conclusions:
Large ventricular fibromas can be resected safely with appropriate surgical planning and an emphasis on debulking. Most children maintain left ventricular function and remain free of recurrent ventricular arrhythmias at follow-up. Extended follow-up is warranted to understand whether patients remain at risk for scar-based ventricular arrhythmias in the future.
