PSAT021 Hemorrhagic Pheochromocytoma Presenting With Cardiogenic Shock: Challenges in Diagnosis and Treatment.
Authors: Zykova Polina , Nguyen Tiffany 
Published: November 2022

Introduction

Pheochromocytomas are rare catecholamine producing tumors with heterogenous clinical presentations. Although acute catecholamine-induced cardiomyopathy is well described in the literature, it is rarely suspected as a cause of cardiogenic shock. Timely diagnosis is challenging and appropriate preoperative treatment is crucial for successful management, as this condition carries a high mortality rate. We describe a case of hemorrhagic pheochromocytoma presenting with refractory hypotension and discuss the difficulties in diagnosis and treatment we encountered.

Case Presentation

A 57-year-old man presented with pallor, severe headache, and hypotension. After administration of intravenous fluids, the patient developed flash pulmonary edema and was found to have acute systolic heart failure. He was taken for emergent left heart catheterization, which did not show any evidence of coronary obstruction. Therefore, he was treated for presumed myocarditis and received stress dose steroids. His condition quickly deteriorated to cardiogenic shock and was further complicated by cardiac arrest. He required extracorporeal membrane oxygenation (ECMO) treatment for 2 days, during which time his systolic blood pressures were extremely labile (47–270 mmHg). CT angiography incidentally noted a 4.3 cm right para-adrenal lesion, at which time urine metanephrines were ordered and noted to be markedly high. Ga-68 DOTATATE scan was negative, however, dedicated abdominal CT revealed a likely hemorrhagic right adrenal mass. With supportive care, the patient's cardiac function recovered by the fifth hospital day, and labetalol was slowly introduced, followed by doxazosin. Sixteen days after the initial presentation, laparoscopic right adrenalectomy was successfully performed. Pathology confirmed pheochromocytoma with few positive cells in a hemorrhagic and necrotic adrenal lesion.

Discussion

Our case underlines several important points in the diagnosis and management of pheochromocytoma.

First, it is crucial to have a high level of suspicion for a catecholamine-secreting tumor in the setting of labile blood pressures and acute cardiomyopathy, especially in cases without evidence of coronary artery or valvular disease. Notably, steroids should be used cautiously, as they may further precipitate catecholamine crisis and have been associated with hemorrhagic pheochromocytomas.

While catecholamine-induced cardiomyopathy is associated with a high mortality rate, the majority of cases are reversible with timely management. Treatment is complex, as standard initial pheochromocytoma management with alpha-blockers and hydration is often precluded due to cardiogenic shock, and vasopressors such as norepinephrine and epinephrine can worsen cardiomyopathy. Furthermore, early initiation of mechanical circulatory support (specifically ECMO) was a life-saving modality, as it facilitated cardiac function recovery and enabled bridging with alpha blockers. Better prognosis is associated with surgery performed after improvement of cardiomyopathy and alpha blockade.

And finally, a multidisciplinary approach is crucial for the successful treatment of pheochromocytoma in emergency situations. Given this rare presentation of pheochromocytoma more, data are needed to define an optimal strategy.

Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.