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    ARTICLE

    Pheochromocytoma and paraganglioma in Fontan patients: Common more than expected

    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… More >

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