Surgical Debulking of Large Ventricular Fibromas in Children

J Thorac Cardiovasc Surg. 2024 May 25:S0022-5223(24)00444-6. doi: 10.1016/j.jtcvs.2024.05.013. Online ahead of print.

Abstract

Objective: This study aims to provide an update on the clinical presentation, diagnostic work-up, operative strategies, and mid-term 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 (IQR 0.8-4.6) and median tumor volume index was 69 milliliters/m2 (IQR 49-169). Tumor distorted the atrioventricular (AV) valve/subvalvar apparatus in 30 (58%) patients and abutted major epicardial coronary arteries in 41 (79%) patients. Surgery was indicated for arrythmia (n=45, 86%), symptoms (n=14, 27%), and/or hemodynamic compromise (n=11, 21%). Tumor was debulked in 34 (65%) patients, including the last 21 patients. Concomitant AV valvuloplasty was performed in 18 patients and ventricular cavity closure in 15 (29%) patients. During a median follow-up of 2.4 years (IQR 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 post-debulking LV ejection fraction did not significantly differ (P=.069), while no patients had signs of outflow tract obstruction, inflow tract obstruction, or > moderate AV 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.

Keywords: debulking; tumor; ventricular fibromas.