An Adrenaline Rush: Management of Pheochromocytoma in Advanced Heart Failure

Abstract

Background Pheochromocytoma is a rare neuroendocrine adrenal tumor characterized by overproduction of catecholamines by chromaffin cells. Clinical manifestations include paroxysms of severe hypertension, palpitations, diaphoresis and headaches. Tumor excision is curative but carries an elevated risk, as removal can lead to sudden fluctuations in catecholamine levels, resulting in life threatening arrhythmias, hemodynamic instability and death. We present a case of perioperative management of a pheochromocytoma in a patient with severe ischemic cardiomyopathy. Case A 47 year old man with hypertension presented with progressive dyspnea on exertion. An echocardiogram revealed a newly depressed ejection fraction of 10-15%. Subsequent coronary angiography demonstrated severe multivessel disease deemed not amenable to percutaneous or surgical intervention. Evaluation of his hemodynamics revealed RA12 RV 59/12 PAP 60/33/44 PCWP 25 MVO2 54% CO/CI 3.2/1.69. Routine CT chest and abdomen demonstrated an incidental 6 cm adrenal mass confirmed to be a pheochromocytoma by metaiodobenzylguanidine scan and urine metanephrines. His perioperative care was managed through a multidisciplinary approach with Advanced Heart Failure, Endocrinology, Oncology, and Surgery. Prior to excision, the patient underwent a 14 day load of phenoxybenzamine. A subclavian intra-aortic balloon pump and Swan Ganz catheter were placed preoperatively for continuous hemodynamic monitoring and support. He subsequently underwent laparoscopic removal without complications. His intra-aortic balloon pump was removed two days postoperatively with slow weaning of inotropic and vasoactive support. Phenoxybenzamine was ultimately discontinued, and he was initiated on goal directed neurohormonal blockade. He was discharged with close outpatient follow up. Discussion This case demonstrates successful management of a pheochromocytoma in a patient with ischemic cardiomyopathy in a decompensated low flow state utilizing preoperative mechanical circulatory support. Successful adrenalectomy was supported by an intra-aortic balloon pump and pre-treatment with alpha-blockade. Pertinent challenges in such cases include timing of surgery and use of temporary support devices. Previous data has been inconclusive regarding timing of excision and perioperative use of percutaneous circulatory support due to risks of anticoagulation. Our case highlights the use of this strategy in select high risk patients.