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Published on: October 20, 2017
Successful surgical management of ventricular fibromas in children
Meena Nathan1, Assunta Fabozzo1, Tal Geva2
1Department of Cardiac Surgery, Boston Children's Hospital and Harvard Medical School, Boston, Mass.
Insights
Surgical resection of large ventricular fibromas in children is effective. This management strategy successfully removed tumors, resolved arrhythmias, and preserved ventricular function in pediatric patients.
Area of Science:
- Pediatric Cardiology
- Cardiac Surgery
- Pediatric Oncology
Background:
- Ventricular fibromas are rare cardiac tumors in children.
- A standardized management approach for large ventricular fibromas is lacking.
- These tumors can cause significant symptoms, including ventricular tachycardia and outflow tract obstruction.
Purpose of the Study:
- To review the institutional experience with surgical resection of ventricular fibromas in pediatric patients.
- To evaluate the efficacy and outcomes of surgical management for this uncommon tumor.
Main Methods:
- Retrospective review of patients diagnosed with ventricular fibromas from July 1990 to June 2013.
- Analysis of clinical records, pathology reports, and follow-up data.
- Focus on patients who underwent surgical intervention.
Main Results:
- Twenty pediatric patients with ventricular fibromas were identified.
- The majority (89%) presented with ventricular arrhythmias.
- All patients achieved successful tumor excision with no recurrence of arrhythmia during follow-up (median 3.3 years).
- No mortality was observed.
Conclusions:
- Surgical resection is a viable and effective treatment for large primary ventricular fibromas in children.
- The procedure leads to excellent short- and intermediate-term ventricular function.
- Surgical management successfully resolves arrhythmic events associated with these tumors.
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.
