ArticleAnnals of pediatric cardiology
Therapeutic challenge in a 5-year-old girl with Gorlin-Goltz syndrome and cardiac fibroma.
Article in Annals of pediatric cardiology. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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6 authors.
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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.
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