SUN-004 Paraganglioma-Induced Catecholamine Crisis Presenting with Cardiomyopathy

Abstract Disclosure: A. Dhulipalli: None. J.K. Sicklick: None. P.S. Ekanayake: None. Background: Catecholamine-secreting tumors like paragangliomas (PGL) are rare neuroendocrine neoplasms that can result in catastrophic cardiometabolic complications if left untreated. Clinical Case: A 61-year-old male with a history of 50-pack year smoking, hypertension, type 2 diabetes and biopsy proven 5.4x3x3.3-cm aortocaval catecholamine-secreting PGL, who previously declined surgical resection, presented to the ER 18 months later. He was found to be in hypertensive emergency and diabetic ketoacidosis (DKA). Lab tests showed blood glucose of 564 mg/dL (ref 70-99), beta-hydroxybutyrate of 17.3 mg/dL (ref <2.8), anion gap of 28 mmol/L (ref 7-15), pH 7.2 (ref 7.33-7.40), troponin of 1403 ng/L (ref <22) and NT-proBNP of 6268 pg/mL (ref <899). Echocardiogram showed an ejection fraction (EF) of 23% without wall motion abnormalities. He had evidence of demand ischemia and congestive heart failure with X-ray evidence of Kerley B lines. Hormonal workup revealed elevated serum epinephrine at 35,390 pg/mL (ref 10-200), norepinephrine at 40,808 pg/mL (ref 80-520), chromogranin A at 862 ng/mL (ref <103), serum metanephrines at 49.5 nmol/L (ref <0.49) and normetanephrines at 39.9 nmol/L (ref <0.8). CT scan showed his PGL had enlarged in the interim to 5.4x4.4x8.1 cm with central necrosis. He was started on insulin drip for DKA and nicardipine drip for hypertensive crisis. He was later switched to alpha blockade (doxazosin, titrated to 8 mg twice a day) followed by beta blockade. After 5 days of doxazosin, EF improved to 56% with resolution of volume overload. Due to high cardiac risk, cardiac catheterization was performed, revealing 90% stenosis of the left anterior descending artery, leading to placement of 2 stents. He was started on Plavix. Six weeks later, he underwent tumor resection. Plavix was held and he was placed on intravenous antiplatelet (P2Y12) inhibitor, cangrelor, until the first surgical incision. Due tumor involvement of the anterior wall of the inferior vena cava, vascular resection with primary inferior vena cava (IVC) repair was required. The estimated blood loss was 2000 mL and his post-operative course was notable for one hypertensive episode. The patient was discharged in good health 7 days postoperatively. Conclusion: Excess catecholamines increase cardiac workload by raising heart rate and blood pressure, which contributes to myocardial ischemia and impaired cardiac function. Alpha before beta-blockade is crucial to prevent unopposed alpha-adrenergic activity. However, surgical resection is the definitive treatment. This case highlights the profound effects of long-standing catecholamine excess on cardiometabolic health and the success of post-operative outcomes when a complex paraganglioma surgery is performed at a tertiary care center with a multidisciplinary team. Presentation: Sunday, July 13, 2025