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Pheochromocytoma is the underlying etiology in 0.1% of hypertensive cases. However, it may be present in up to 5.7% of patients with neurofibromatosis I (NF1). The burst of catecholamines inherent in pheochromocytoma has significant effects on the mechanical and electrical activity of the myocardium. Different theories have been postulated for myocardial stunning in patients with pheochromocytoma that include microvascular spasm, impaired fatty acid metabolism, increased production of oxygen-derived free radicals and dynamic left ventricular mid-cavity obstruction. QT interval prolongation is seen in 16% to 35% of patients with pheochromocytoma. Takotsubo cardiomyopathy (TS) is now being increasingly identified and it may be responsible for up to 40% of cases of acute catecholamine cardiomyopathy. These manifestations may sometimes precede or cloud the typical triad of a headache, sweating, and tachycardia.  We herein present a case of a 42-year-old female with a unique combination of QT prolongation, torsades de pointes, and TS caused by pheochromocytoma in the background of NF1. All these complications are potentially reversible with the removal of the underlying adrenal tumor, underscoring the importance of a high suspicion for pheochromocytoma in patients with NF1.

Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma