ESA-SRB-AOTA 2019

A phaeochromocytoma crisis presenting with a severe takotsubo-like cardiomyopathy treated with ECMO (#578)

Emma Boehm 1 , Joshua Hawson 2 , Subodh Joshi 2 , Spiros Fourlanos 1 , Christopher J Yates 1
  1. Dept Diabetes and Endocrinology, Royal Melbourne Hospital, Melbourne, VIC, Australia
  2. Dept Cardiology, Royal Melbourne Hospital, Melbourne, Vic, Australia

Introduction: Phaeochromocytoma crises can be precipitated by medications and can rarely present as a takotsubo-like cardiomyopathy that poses challenging haemodynamic management issues. 

Case: A 39-year-old female presented with severe headache. Her initial blood pressure was 180/90mmHg. She was treated for a presumed migraine with morphine, metoclopramide, and phenothiazines. Following an episode of syncope, an ECG showed changes concerning for ST-elevation myocardial infarction. Urgent coronary angiography showed normal coronary arteries and a reverse takotsubo cardiomyopathy with ejection fraction of 5-10% (normal >50%). The patient developed cardiogenic shock refractory to inotropic support and was commenced on extracorporeal membrane oxygenation (ECMO). Plasma metanephrines were sent, however in the interim an abdominal ultrasound revealed an 8cm mass abutting the right upper renal pole.  The patient was commenced on a phentolamine infusion for a presumptive diagnosis of a phaeochromocytoma crisis, potentially precipitated by metoclopramide. She was transitioned to phenoxybenzamine and metoprolol and ECMO ceased on day 9. A repeat echocardiogram showed normal left ventricular function. A Gallium Octreotate PET/CT revealed a large avid left adrenal mass with no metastasis. A left adrenalectomy was performed 6 weeks later with histopathology confirming a phaeochromocytoma.

Discussion:Commonly prescribed medications can exacerbate phaeochromocytoma crises, which typically present with paroxysms of headache, palpitations and chest pain. A takotsubo-like acute catecholamine-mediated cardiomyopathy has also been described1. Stunning of myocardial fibres occurs as catecholamine excess drives massive concentrations of calcium into the sarcoplasmic reticulum, reducing mitochondrial energy production1. ECMO is a solution to the unique haemodynamic challenges of providing alpha- and then beta-adrenoceptor blockade to a patient with cardiogenic shock to allow for recovery of myocardial function2.

Conclusion:Phaeochromocytoma crisis should be considered in the differential diagnosis for a takotsubo-like cardiomyopathy. In this case ECMO was successfully used to overcome the challenge of providing adrenoreceptor blockade to a patient in cardiogenic shock.

  1. Kounatiadis, P., Kolettas, V., Megarisiotou, A. and Stiliadis, I. (2013). Cardiomyopathy due to pheochromocytoma. Herz, 40(1), pp.139-143.
  2. Banfi, C., Juthier, F., Ennezat, P., de Saint Denis, T., Carnaille, B., Leteurtre, E., Prat, A. and Vincentelli, A. (2012). Central Extracorporeal Life Support in Pheochromocytoma Crisis. The Annals of Thoracic Surgery, 93(4), pp.1303-1305.