Moore Lily M, Knudson Jarrod D
Department of Pediatrics, University of Mississippi Medical Center, Jackson, MS, USA.
Division of Pediatric Critical Care, University of Mississippi Medical Center, Jackson, MS, USA.
Cardiol Young. 2019 Aug;29(8):1112-1114. doi: 10.1017/S1047951119001628. Epub 2019 Jul 9.
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.
嗜铬细胞瘤/副神经节瘤是一种极其罕见的肿瘤，被认为与紫绀型先天性心脏病有关。这种关联被认为是慢性低氧血症的结果（安东尼奥等人，《西班牙心脏病学杂志（英文版）》2017年；70: 673 - 675；福尔杰等人，《循环》1964年；29: 750 - 757；奥波托夫斯基等人，《临床内分泌与代谢杂志》2015年；100: 1325 - 1334）。我们报告了两例在4年期间发生于左心发育不全综合征患者的副神经节瘤病例，这两名患者分别在2岁和4岁时接受了Fontan手术。基于非常少量的先天性心脏病报告病例，肿瘤发生机制尚不清楚。必须继续报告与先天性心脏病相关的病例，以便我们能更多地了解这一实体的发病机制和流行病学。
