Cardiogenic shock secondary to phaeochromocytoma: adrenalectomy on multiple mechanical circulatory support devices

Abstract

Introduction Phaechromocytoma is a neuroendocrine tumour of the chromaffin cells of the adrenal medulla. Tumours secrete catecholamines and typically present with hypertensive crisis or, in rare circumstances, cardiogenic shock. Case Presentation A 33 year old male presented with hypertensive crisis and fulminant cardiogenic shock. He required mechanical ventilation for hypoxaemia secondary to acute pulmonary oedema. A CT aortogram was performed for presumed aortic dissection which revealed a necrotic, enhancing adrenal mass. Due to severe left ventricular dysfunction, he was referred to our hospital for mechanical circulatory support (MCS). Peripheral veno-arterial extracorporeal membrane oxygenation (VA-ECMO) was established in the referral hospital and he was transferred to our intensive care unit. An Impella-CP was then inserted to offload an increasingly dilated left ventricle. Hypertensive crises continued to occur despite maximum medical therapy, and it was determined the only viable option to control these paroxysms of hypertension was to perform adrenalectomy. Surgery was successfully performed whilst on dual MCS devices (VA-ECMO and Impella). Preoperatively the patient had intravenous alpha and beta adrenoceptor blockade and fluid status was optimised to address the chronic circulating volume depletion. Glucose and electrolyte imbalances were corrected. Intra-operative management focused on blood pressure control, heart rate and arrhythmia control. Agents that induce catecholamine release were avoided and tumour manipulation was minimised. Hypotension after tumour removal was anticipated and developed relatively quickly requiring infusions of noradrenaline, adrenaline and vasopressin to support an adequate arterial pressure. His post-operative recovery was initially favourable and myocardial function had improved to the point of planning ECMO explanation. Despite cardiovascular stability the patient suffered a catastrophic intracranial haemorrhage and treatment was withdrawn. Discussion Cardiogenic shock secondary to phaeochromoctyoma can be difficult to manage and has high mortality. The evolution of short to medium term MCS devices has provided clinicians with additional therapeutic options to manage refractory cases of this uncommon cause of cardiogenic shock. The authors hope this case will add to an expanding library of practice of using MCS in phaeochromocytoma.