Fellow - Cardiovascular disease Allegheny General Hospital Allegheny General Hospital Pittsburgh, Pennsylvania, United States
Disclosure(s):
Aakash Tuli, MD: No financial relationships to disclose
Background: Rare adrenal masses can cause life-threatening cardiovascular crises, often triggered by stressors. This case outlines a bee sting leading to cardiogenic shock and cardiac arrest, necessitating VA-ECMO rescue. The diagnostic process and multidisciplinary care underscore the challenges in managing these dramatic and complex presentations.
Methods: A 39-year-old female with a known bee sting allergy presented to the emergency department (ED) after a bee sting. She self-administered an EpiPen prior to arrival. In the ED, she was treated with methylprednisolone, diphenhydramine, and famotidine for presumed anaphylaxis and discharged after symptom resolution.
Shortly after discharge, she returned with severe nausea, vomiting, and inability to tolerate oral intake. Laboratory evaluation revealed hyperglycemia and mild anion gap metabolic acidosis. Suspected diabetic ketoacidosis (DKA) or hyperosmolar hyperglycemic state (HHS) prompted admission for insulin therapy. A CT scan showed a distended, acalculous gallbladder, an adrenal mass, and findings of ileitis/colitis.
Her condition deteriorated rapidly, culminating in recurrent pulseless electrical activity (PEA) arrests and cardiac standstill. Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and intra-aortic balloon pump (IABP) were initiated. Echocardiography showed severe biventricular dysfunction with a left ventricular ejection fraction (LVEF) of 10-15%. Shortly thereafter, she required escalation to an Impella 5.5 device. Coronary angiography was normal.
Within 48 hours, echocardiography showed complete recovery of biventricular function (LVEF > 75%). VA-ECMO was decannulated, and Impella support was discontinued. Imaging revealed an enlarged adrenal mass (4.7 cm), raising suspicion for a functioning lesion, with elevated 24-hour urinary vanillylmandelic acid (VMA) levels suggesting pheochromocytoma.
Outcome: We could not definitively differentiate between catecholamine release due to stress and exogenous epinephrine administration during cardiopulmonary resuscitation (CPR) versus a pheochromocytoma. A multidisciplinary team comprising cardiac intensivists, advanced heart failure specialists, endocrinologists, and surgeons was convened. After thorough discussion, the team decided to proceed with surgical excision of the adrenal mass. To optimize perioperative outcomes, the patient was pretreated with alpha-blockers to achieve adequate blood pressure control, minimizing the risk of hypertensive crises during surgery. Cardiac anesthesia was integrated into the perioperative plan to ensure comprehensive hemodynamic monitoring and management.
The patient underwent an open right adrenalectomy, which was completed without intraoperative complications. Post-surgical histopathological examination confirmed the diagnosis of pheochromocytoma. Immunohistochemical analysis demonstrated strong positivity for synaptophysin, chromogranin A, and S100, consistent with the neuroendocrine origin of the tumor. Inhibin staining was negative, and the Ki-67 index was approximately 1%, indicative of a low proliferative rate and suggesting a less aggressive behavior of the tumor.
The patient’s postoperative recovery was uneventful. She experienced no significant complications and was discharged home in stable condition.
Conclusion: This case illustrates the catastrophic cardiovascular effects of unrecognized pheochromocytoma, including cardiac arrest, successfully managed with VA-ECMO and mechanical circulatory support. This case underscores the importance of a multi-disciplinary team approach for complex decision making in patient with unexplained cardiovascular collapse.