Anesthetic management of pheochromocytoma and paraganglioma for patients with Fontan circulation: a case series

Abstract

Fontan circulation is an iatrogenic definitive palliative condition for patients with single ventricle physiology. The patient with Fontan circulation can be sensitive and vulnerable to change systemic vascular resistance (SVR), pulmonary vascular resistance (PVR), and inotropy. Pheochromocytoma and paraganglioma (P-PGL) are neuroendocrine tumors with catecholamine hypersecretion. The number of patients with Fontan circulation and P-PGL is expected to increase because survival rate of the patients is improved and P-PGL have a tendency to develop under cyanotic heart disease [1, 2]. Chronic hypoxia exposure followed by aberrant activation of common hypoxia cellular pathways and hypoxia-inducible factors in chromaffin cells appear to mechanism for the pathogenesis of P-PGL [1].