Successful surgical management of ventricular fibromas in children

Objectives A uniform management strategy has not been established for the treatment of large ventricular fibromas identified in children. The goal of the present study was to review our experience with surgical resection of this uncommon tumor. Methods We performed a retrospective review of all patients with a diagnosis of ventricular fibromas who had undergone surgical intervention at our institution from July 1990 to June 2013. The clinical records and pathology reports were reviewed and available follow-up data obtained. Results A total of 20 patients had a ventricular fibroma. The median age at presentation was 4 years (range, 5 months to 12 years). Of these, 18 (89%) presented with ventricular tachycardia (n = 15) or frequent ectopy (n = 3), and 1 (6%) with outflow tract obstruction; 1 (6%) was asymptomatic and diagnosed during routine evaluation for a murmur. One patient had undergone previous ablation, and one had had an automated internal cardiac defibrillator implanted for ventricular tachycardia control. Before tumor resection at our institution, 1 patient had been listed for transplantation at an outside institution and 1 had undergone previous extracardiac Fontan without tumor resection. All patients underwent successful tumor excision with no recurrence of ventricular arrhythmia during a median follow-up period of 3.3 years (range, 1 month to 14.7 years). One patient underwent planned staged tumor resection. No patient died. Conclusions Primary ventricular fibromas, even when very large, can be managed by surgical resection, with excellent short- and intermediate-term ventricular function and relief of arrhythmic events