Hostname: page-component-76fb5796d-vfjqv Total loading time: 0 Render date: 2024-04-27T02:11:13.520Z Has data issue: false hasContentIssue false
  • English
  • Français

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: jknudson@umc.edu
Get access Rights & Permissions [Opens in a new window]

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 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Folger, GM, Roberts, WC, Mehrizi, A, et al. Cyanotic malformations of the heart with pheochromocytoma. Circulation 1964; 29: 750–757. doi: 10.1161/01.cir.29.5.750. CrossRefGoogle ScholarPubMed
Opotowsky, AR, Moko, LE, Ginns, J, et al. Pheochromocytoma and paraganglioma in cyanotic congenital heart disease. J Clin Endocrinol Metab 2015; 100: 1325–1334. doi: 10.1210/jc.2014-3863.CrossRefGoogle ScholarPubMed
Yamamoto, K, Namba, N, Kubota, T, et al. Pheochromocytoma complicated by cyanotic congenital heart disease: a case report. Clin Pediatr Endocrinol 2016; 25: 59–65. doi: 10.1297/cpe.25.59.CrossRefGoogle ScholarPubMed
Antonio, IPD, Cantador, JR, García, AEG, Ruiz, JMO, et al. Prevalence of neuroendocrine tumors in patients with cyanotic congenital heart disease. Revista Española de Cardiología (English Edition) 2017; 70: 673–675. doi: 10.1016/j.rec.2016.09.036.CrossRefGoogle Scholar
Cheung, YW, Spevack, DM. Single left ventricle and pheochromocytoma. Congenit Heart Dis 2008; 3: 355–358. doi: 10.1111/j.1747-0803.2008.00183.x.CrossRefGoogle ScholarPubMed
Online Mendelian Inheritance in Man, OMIM (TM). Johns Hopkins University, Baltimore, MD. MIM 603297. 04/09/2019. https://www.omim.org/entry/603297.Google Scholar
Favier, J, Gimenez-Roqueplo, AP. Pheochromocytomas: the (pseudo)-hypoxia hypothesis. Best Pract Res Clin Endocrinol Metab 2010; 24; 957–968. doi: 10.1016/j.beem.2010.10.004.CrossRefGoogle ScholarPubMed
Kita, T, Imamura, T, Date, H, et al. Two cases of pheochromocytoma associated with tetralogy of Fallot. Hypertens Res 2003; 26: 433–437. doi: 10.1291/hypres.26.433.CrossRefGoogle ScholarPubMed