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Barrie S. Rich, Tracy-Ann Moo, Sharayne Mark, Theresa Scognamiglio, Mark S. Pecker, Irina Sobol, Gina M. LaRocca, Thomas J. Fahey, Sympathetic Paraganglioma in a Patient with Unrepaired Tetralogy of Fallot: A Case Report and Review of the Literature, The Journal of Clinical Endocrinology & Metabolism, Volume 98, Issue 1, 1 January 2013, Pages 7–12, https://doi.org/10.1210/jc.2012-1969
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Abstract
Paragangliomas are a type of neuroendocrine tumor that has been reported to be present in patients with cyanotic congenital heart disease. This report documents the first case of a patient with successful resection of a sympathetic paraganglioma in the setting of unrepaired tetralogy of Fallot, the most common cause of cyanotic heart disease, with pulmonary atresia.
We present a 33-yr-old woman with hypertensive crises from a paraganglioma who presented for surgical resection.
The patient's preoperative workup was consistent with a functioning sympathetic paraganglioma. Preoperative transesophageal echocardiogram displayed normal ventricular function, moderate-severe right ventricular hypertrophy, severe right ventricular hypertension, an overriding aorta, bidirectional shunting, pulmonary atresia, and aortopulmonary collaterals.
The patient underwent a successful laparoscopic resection of a functioning 7-cm paraganglioma after careful preoperative preparation and intraoperative monitoring. Pathology returned as a well-defined, partially hemorrhagic mass measuring 7.0 × 4.5 × 4.5 cm adjacent to and compressing the adrenal gland.
Surgical resection of paraganglioma tumors in rare patients such as this one is appropriate; however, surgery requires meticulous perioperative management with a multidisciplinary approach. Future studies are needed to determine whether there is a link between neuroendocrine tumors and cyanotic congenital heart disease.