Abstract 12938: An Unusual Case of Malignant Hypertension and Stress Cardiomyopathy Originating From the Urinary Bladder

Abstract

A 39-year-old man with hypertension presented for an elective knee surgery. Within 15 minutes of induction of anesthesia, he developed malignant hypertension and acute hypoxia. Surgery wasaborted and he was subsequently transferred to our facility. High-sensitivity troponin was elevated. Chest X-ray demonstrated bilateral hilar opacities, consistent with pulmonary edema.Transthoracic echocardiography demonstrated mildly depressed left ventricular function with hypokinesis of the septum and inferolateral wall. Cardiac catheterization showed normal filling pressures and no coronary artery disease. Initial work up for secondary hypertension, including Duplex Doppler ultrasonography of renal artery and serum aldosterone level were unrevealing. Because of high index of suspicion, additional workup was pursued for adrenal hypertension. Hewas found to have severely elevated plasma normetanephrine level (36 nmol/L) and 24-hrurinary normetanephrine of 8,990 mcg while plasma metanephrine and epinephrine levels were within normal limits. Computed tomography of abdomen showed no adrenal mass but revealed contrast-enhancing mass in the bladder wall with surrounding lymphadenopathy, suggestive of metastatic paraganglioma. He underwent genetic evaluation which identified a heterozy gouspathogenic variant in the succinate dehydrogenase subunit B (SDHB) gene. He was treated withalpha- and beta-blockade and underwent partial cystectomy with lymph node dissection. Follow-up Gallium-68 DOTATATE scan was positive for uptake in several lymph nodes withsubsequent successful lymph node dissection. He is now asymptomatic. Discussion: This case illustrates the importance of a complete history in the evaluation of hypertension inyoung people. The presence of labile hypertension and acute cardiomyopathy which isprecipitated by stress or noncardiac surgery should prompt an evaluation of pheochromocytoma.The isolated elevation in plasma and urinary normetanephrine with normal plasma/urinarymetanephrine level is consistent with extra-adrenal production of catecholamines, which iscommon in the presence of SDHB mutation.