Mi Kyoung Song1, Gi Beom Kim1, Eun Jung Bae1, Young Ah Lee1, Hyun-Young Kim2, Seung-Kee Min3, Jung Hee Kim4, Jae-Kyung Won5
Congenital Heart Disease, Vol.13, No.4, pp. 608-616, 2018, DOI:10.1111/chd.12625
Abstract Objective: Pheochromocytoma and paraganglioma (extra-adrenal pheochromocytoma) are rare
neuroendocrine tumors that arise from the neuroendocrine cells. Chronic hypoxia is known as a
possible cause, and a strong link between cyanotic congenital heart disease and these tumors has
been reported. However, reports of phechromocytoma/paraganglioma in Fontan patients were
scarce. We herein report seven cases of phechromocytoma/paraganglioma after Fontan operation
at a single tertiary center.
Methods: We retrospectively reviewed medical records and imaging studies who diagnosed as
phechromocytoma/paraganglioma after Fontan operation in Seoul National University Children’s
Hospital.
Results: Seven patients were identified during follow-up after Fontan operation, and the prevalence was 2.5%… More >
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