title: Sympathetic paraganglioma in a patient with unrepaired tetralogy of Fallot: a case report and review of the literature.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  We present a 33-yr-old woman with hypertensive crises from a paraganglioma who presented for surgical resection.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Surgery, Weill Cornell Medical Center, New York, New York 10065, USA.
authors.forename: Barrie S
authors.initials: BS
authors.lastname: Rich
authors.forename: Tracy-Ann
authors.initials: TA
authors.lastname: Moo
authors.forename: Sharayne
authors.initials: S
authors.lastname: Mark
authors.forename: Theresa
authors.initials: T
authors.lastname: Scognamiglio
authors.forename: Mark S
authors.initials: MS
authors.lastname: Pecker
authors.forename: Irina
authors.initials: I
authors.lastname: Sobol
authors.forename: Gina M
authors.initials: GM
authors.lastname: LaRocca
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Fahey
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D000328:Adult; D005240:Feasibility Studies; D005260:Female; D005728:Ganglia, Sympathetic / Q000473:pathology / Q000601:surgery; D006801:Humans; D010235:Paraganglioma / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D011860:Radiography, Abdominal; D013771:Tetralogy of Fallot / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D014057:Tomography, X-Ray Computed