Case of the broken hearted

Phaeochromocytomas are rare neuroendocrine tumours which, usually present with headache, diaphoresis, episodic tachycardia and hypertension. Takotsubo-pattern cardiomyopathy is an unusual initial presentation of phaeochromocytomas, however has been reported1. Approximately 10% of phaeochromocytomas present with cardiomyopathy. It is associated with a high mortality risk and early intervention with surgery is the key to recovery. We report a case of a previously healthy, 68 year old man who presented with symptoms of left ventricular failure, and feeling anxious. Physical examination revealed signs of left ventricular failure, with a normal blood pressure and heart rate. A transthoracic echocardiogram (TTE) revealed dilated left ventricle with severe global hypokinesis and a left ventricular ejection fraction (LVEF) of 15%. A CTPA performed to exclude pulmonary embolism revealed an incidental right sided adrenal lesion measuring 3.9 X 3.4 X 3.7 cm. Subsequently a dedicated abdominal CT confirmed the adrenal lesion. Biochemistry revealed elevated 24 hour urine catecholamines and metanephrines and an elevated plasma metanephrines. Subsequently he proceeded to have adrenalectomy. Pre-operatively the patient was managed with alpha and beta blocking agents for 3 weeks prior to surgery. Post-operative course was uneventful and histopathology confirmed right sided phaeochromocytoma. TTE performed 12 months post-operatively showed a significant improvement in his LVEF to 40 % with subsequent TTE showing maintenance of LVEF. This case demonstrates highlights the unusual presentations of phaeochromocytomas and that early recognition and early intervention with surgery is the key to recovery to avoid catastrophic cardiac events.