Cyanotic heart disease: "low altitude" risk for carotid body tumor?

A young woman with hypoplastic right heart syndrome developed a carotid body tumor at age 28. High altitude habitation is known to predispose to hyperplastic and neoplastic carotid bodies; emphysema and congenital cyanotic heart disease have recently been shown to induce hyperplasia of this oxytrophic tissue. Therefore, a link between congenital cyanosis and carotid body tumor is suggested by this patient. Carotid bodies are nonchromaffin paraganglionic analogues of the adrenal medulla. Congenital cyanosis has an association with pheochromocytoma. In the cyanotic milieu carotid body tumor may be a histological analogue of pheochromocytoma.