A 70-year-old female with a recent history of anxiety and completely resolved Takotsubo cardiomyopathy, presented with chest pain and mild hypertension (SBP 154mmHG) associated with anterior ST changes and troponin of 2052ng/L (RR <16). Coronary angiography was normal and transthoracic echocardiogram demonstrated an EF of 27%. She was treated with oral metoprolol for a presumed recurrent Takotsubo’s cardiomyopathy.
Within 24 hours she developed acute cardiogenic shock with bradycardia requiring ICU support and potential transfer for extracorporeal membrane oxygenation. A CT to investigate aortic dissection was negative but a 6cm right adrenal mass was identified.
Endocrine were consulted and plasma metanephrines, cortisol and ACTH, and an aldosterone/renin ratio were sent. Due to high clinical suspicion for pheochromocytoma, patient was commenced on alpha blockade with oral prazosin and all beta-blockers were strictly withheld.
All plasma metanephrines were significantly elevated; metadrenaline 36,000pmol/L (RR 30-540), normetadrenaline 20,000pmol/L (RR 13-1600) and 3 methoxy Tyramine 682pmol/L (RR<120).
Cortisol and ACTH were also elevated at 1649nmol/L (RR 80-480nmol/L) and 15.7pmol/L (RR <14) which raised suspicion of an ACTH-producing phaeochromocytoma. A 1mg overnight dexamethasone suppression test was also abnormal, with post-dexamethasone cortisol 970nmol/L. Clinically the patient was not cushingoid.
Two weeks after the acute presentation, her cardiac function improved to an EF of 55%. ACTH and cortisol also normalised (3.2pmol/L and 279nmol/L respectively), suggesting the significant hypercortisolaemia represented an acute stress response.
Four weeks after the initial presentation, she underwent a laparoscopic right adrenalectomy. Tumour cells stained positive for synaptophysin and did not have cytological features of malignancy with total PASS score of zero. Total tumour weight was 84.1g. Post-operative echocardiogram demonstrated reduction in EF to 45%, suggesting effects of intraoperative catecholamine release, despite alpha and beta blockade.
This case demonstrates the importance of high clinical suspicion for pheochromocytomas or paragangliomas in Takotsubo or hypertension induced cardiomyopathy.