Presentation of an Unsuspected Pheochromocytoma as Acute Aortic Valvular Insufficiency and Diabetes Mellitus Type 2

Abstract

We report the uncommon presentation of an unsuspected pheochromocytoma in a 76-year-old woman. Two years after surgery for acute aortic valve insufficiency and a diagnosis of diabetes, the patient presented with postural headaches, orthostatic dizziness, and palpitations. Physical examination confirmed intractable hypertension, orthostatic hypotension, and congestive heart failure secondary to progressive dilated cardiomyopathy. Based on her symptomatology, examination findings, and progressive dilated cardiomyopathy despite aortic valve replacement, a diagnosis of a pheochromocytoma was entertained. Twenty-four-hour urinary studies confirmed the presence of a catecholamine producing tumor. A magnetic resonance image revealed the presence of a 4.6-cm left adrenal mass, which characteristically enhanced on T2 imaging. After medical stabilization with phenoxybenzamine, she underwent a left robotic adrenalectomy. Histology confirmed a diagnosis of a pheochromocytoma. Two weeks later, she was normoglycemic and normotensive off all medications. The pheochromocytoma is a rare catecholamine-producing tumor, which can be lethal unless recognized and appropriately treated. A high degree of clinical suspicion is necessary, especially in the presence of confounding disease processes. A pheochromocytoma should be considered in the differential diagnosis of acute aortic valvular insufficiency in the setting of orthostatic hypotension, intractable hypertension, and newly diagnosed diabetes. Learning Objectives• Recall the presenting clinical features of this patient when seen 2 years after replacement of an insufficient aortic valve, emphasizing cardiovascular abnormalities. • Point out the lethality of unrecognized pheochromocytoma and diagnostic measures that may permit timely surgical treatment. • Explain relationships among hypersecretion of catecholamines by a pheochromocytoma, cardiovascular disease, and type 2 diabetes. P heochromocytoma presenting as aortic valve insufficiency, progressive dilated cardiomyopathy, orthostatic hypotension, and diabetes is uncommon. We report an unusual case of a 76-year-old woman with a recent history of acute aortic valvular insufficiency and diabetes mellitus type 2 who presented with congestive heart failure, orthostatic hypotension, and was subsequently found to have an unsuspected pheochromocytoma. She had rapid resolution of her intractable orthostatic hypotension, congestive heart failure, and diabetes after the surgical removal of the tumor. CASE REPORTA 76-year-old woman was first hospitalized 2 years before the present illness for the surgical repair of acute aortic valve insufficiency. At that time, she was simultaneously diagnosed with type 2 diabetes requiring oral medications. Her medical history was notable for uncontrolled hypertension. An echocardiogram revealed severe acute aortic valvular insufficiency with evidence of normal left ventricular size and function. Cardiac catheterization demonstrated no evidence of coronary artery disease.