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Two rare cases of paraganglioma in patients with Fontan physiology

Part of: Functionally Univentricular Heart

Published online by Cambridge University Press:  09 July 2019

Lily M. Moore  and
Jarrod D. Knudson
Lily M. Moore
Affiliation:
Department of Pediatrics, University of Mississippi Medical Center, Jackson, MS, USA
Jarrod D. Knudson*
Affiliation:
Division of Pediatric Critical Care, University of Mississippi Medical Center, Jackson, MS, USA
*
Author for correspondence: Jarrod D. Knudson, MD PhD, Associate Professor of Pediatrics, Chief of Pediatric Critical Care, University of Mississippi Medical Center, 2500 N. State Street Jackson, MS 39216, USA. Tel: 601 815 8173; Fax 601 984 5982; E-mail: [email protected]
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Abstract

Pheochromocytoma/paraganglioma is an exceedingly rare tumour, thought to share an association with cyanotic CHD. This association is thought to be a result of chronic hypoxaemia (Antonio et al, Revista Española de Cardiología (English Edition) 2017; 70: 673–675; Folger et al, Circulation 1964; 29: 750–757; Opotowsky et al, J Clin Endocrinol Metab 2015; 100: 1325–1334) We report two cases of paraganglioma over a 4-year period in patients with hypoplastic left heart syndrome who had undergone Fontan completion by ages 2 and 4. Based on a very small number of reported cases of CHD, the mechanism of tumourigenesis is unclear. It is imperative that cases associated with CHD continue to be reported so that we may learn more about the pathogenesis and epidemiology of this entity.

Keywords

Pheochromocytoma/paragangliomacyanotic CHDhypoplastic left heart syndromeFontan palliationhypoxaemiatumourigenesis
Type
Brief Report
Information
Cardiology in the Young , Volume 29 , Issue 8 , August 2019 , pp. 1112 - 1114
Copyright
© Cambridge University Press 2019 

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