Therapeutic challenge in a 5-year-old girl with Gorlin-Goltz syndrome and cardiac fibroma.
This case report describes partial resection of a large cardiac fibroma in a 5-year-old girl with Gorlin-Goltz syndrome after infection-associated ventricular fibrillation, with no sustained ventricular arrhythmia detected by a loop recorder over 9 months.
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This case report describes partial resection of a large cardiac fibroma in a 5-year-old girl with Gorlin-Goltz syndrome after infection-associated ventricular fibrillation, with no sustained ventricular arrhythmia detected by a loop recorder over 9 months.
Research significance
The reported case provides preliminary evidence that partial cardiac fibroma resection may help prevent recurrent malignant ventricular arrhythmia when complete resection is not selected; whether tumor debulking causally reduces recurrence, and which patients benefit, remains an inference requiring larger and longer studies.
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.