Management of Pheochromocytoma in a Patient With Severe Aortic Stenosis Undergoing Transcatheter Aortic Valve Replacement

Abstract

Background: There are few guidelines for the management of patients with both significant cardiac valvular disease and pheochromocytoma. Resection of pheochromocytoma is challenging for surgeons and anesthesiologists due to the potential for sudden releases of catecholamines causing significant hemodynamic effects. The cornerstone of perioperative management of pheochromocytoma involves alpha blockade to prevent the potential for hypertensive crisis from unopposed alpha-adrenergic vasoconstriction. Achieving adequate adrenergic blockade is challenging in patients with aortic stenosis due to the risks of decreased afterload precipitating myocardial ischemia and bradycardia leading to diminished cardiac output [1]. It is difficult to determine whether to proceed first with transcatheter aortic valve replacement or pheochromocytoma resection in patients with both conditions. Clinical Case: An 83-year-old woman with a past medical history of type 2 diabetes, essential hypertension, coronary artery disease, and severe aortic stenosis was found to have a left adrenal mass (10.7 cm) on coronary CTA during pre-operative evaluation for transcatheter aortic valve replacement. She had significantly elevated total urine metanephrines of 16,237 ug/24 hr (n 140–785 ug/24 hr); other hormonal workup was unremarkable. A multi-disciplinary team of interventional cardiology, anesthesiology, and endocrinology specialists determined the safest management plan involved proceeding with transcatheter aortic valve replacement, after adequate alpha and beta blockade and prior to pheochromocytoma resection, to avoid potential for severe heart failure. Due to the potential for unpredictable catecholamine release intra and peri-operatively, the patient was at high risk for hypertensive crisis and mortality. Her home medications of metoprolol 50 mg twice daily, spironolactone 25 mg daily, and verapamil 120 mg daily were continued. She was started on treatment with phenoxybenzamine 10 mg in morning and 10 mg in evening. The phenoxybenzamine dose was titrated to 10 mg in the morning and 20 mg in the evening to achieve a consistent systolic blood pressure of less than 130 mm Hg. She successfully underwent transcatheter aortic valve replacement and remained hemodynamically stable. She was discharged with plan for surgical resection of pheochromocytoma six weeks after her transcatheter aortic valve replacement. Conclusions: This case describes a successful management strategy for achieving alpha blockade in an elderly patient with pheochromocytoma and severe aortic stenosis. Reference: [1] Saran JS, Moalem J, Schoeniger L, Tzimas K. Perioperative Management of Pheochromocytoma Resection in a Patient With Severe Aortic Stenosis. J Cardiothorac Vasc Anesth. 2018 Dec;32(6):2712–2715.