Therapeutic challenge in a 5-year-old girl with Gorlin-Goltz syndrome and cardiac fibroma.
This report describes a 5-year-old girl with Gorlin-Goltz syndrome, a large cardiac fibroma, and infection-associated ventricular fibrillation who underwent partial tumor resection and had no sustained ventricular arrhythmia recorded over nine months of postoperative monitoring.
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This report describes a 5-year-old girl with Gorlin-Goltz syndrome, a large cardiac fibroma, and infection-associated ventricular fibrillation who underwent partial tumor resection and had no sustained ventricular arrhythmia recorded over nine months of postoperative monitoring.
Research significance
The case provides preliminary evidence that partial resection is feasible and was followed by an arrhythmia-free monitored interval; it supports—but does not establish—the hypothesis that reducing cardiac fibroma burden may lower recurrent ventricular fibrillation risk when complete resection is impractical.
Source abstract
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.