Key result
Partial resection of a large cardiac fibroma eliminates sustained ventricular arrhythmias through 9 months.
Why the study?
Cardiac fibromas in Gorlin-Goltz syndrome are rare and pose a high risk of malignant arrhythmias with no well-established standard management.
Does partial surgical resection prevent the recurrence of ventricular fibrillation in a pediatric patient with Gorlin-Goltz syndrome and a large cardiac fibroma?
Case Report (n=1)
No
Does partial surgical resection prevent the recurrence of ventricular fibrillation in a pediatric patient with Gorlin-Goltz syndrome and a large cardiac fibroma?
Partial tumor resection can be an effective and safe secondary prevention strategy for ventricular fibrillation in pediatric patients with large cardiac fibromas and Gorlin-Goltz syndrome when complete resection is anatomically prohibitive.
Gorlin-Goltz syndrome (GGS) is a rare hereditary autosomal dominant condition. Cardiac fibromas are one of the clinical manifestations of this disease. The presence of these benign heart tumors may lead to life-threatening complications. We present the case of a 5-year-old girl with GGS and a large cardiac fibroma who had ventricular fibrillation during respiratory tract infection. Several options of treatment were considered in the secondary prevention of cardiac arrest. A partial resection of the tumor was performed to prevent recurrence of malignant arrhythmia. The loop recorder implanted postoperatively didn't record any episodes of sustained ventricular arrhythmia in a 9-month follow-up. In conclusion, cardiac fibromas, especially with the presence of infections, increase risk of ventricular arrhythmias. Partial resection of tumor can be effective and relatively safe in the secondary prevention of ventricular fibrillation in patients with large cardiac fibromas with GGS.
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Pskit et al. (2026) conducted a case report in Gorlin-Goltz syndrome and cardiac fibroma (n=1). Partial resection of cardiac fibroma was evaluated on Recurrence of sustained ventricular arrhythmia. Partial resection of a large cardiac fibroma prevented the recurrence of sustained ventricular arrhythmias over a 9-month follow-up in a 5-year-old girl with Gorlin-Goltz syndrome.
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