Key result
Surgical resection after alpha-blockade completely resolves symptoms and normalizes LVEF in extra-adrenal pheochromocytoma.
Case Report (n=1)
Highlights the rare presentation of extra-adrenal pheochromocytoma as inverse takotsubo-pattern cardiomyopathy, emphasizing the need to consider catecholamine-secreting tumors in atypical cardiomyopathy presentations.
May prompt pheochromocytoma evaluation in atypical cardiomyopathy; single case leaves open whether resection routinely normalizes EF.
Pheochromocytoma is a rare catecholamine-secreting tumour that is typically located in the adrenal medulla or along the sympathetic ganglia. The typical symptoms are episodic in nature and include tachycardia, sweating and headache. These tumours can present as transient, reversible cardiomyopathy similar to takotsubo cardiomyopathy (TCM). TCM is characterised by transient hypokinesis of the left ventricular apex and is typically induced by emotional stress. We describe the case of a 26-year-old woman with a medical history significant for headaches who presented initially to her family physician with nausea, vomiting, headache and hypertension. She was started on lisinopril 10 mg daily. One week later, she presented to the emergency department with substernal severe chest pressure. Her troponin level was elevated. Coronary angiogram showed normal coronary arteries and left ventriculogram showed inverse TCM pattern. Serum catecholamines were very elevated confirming pheochromocytoma. She was successfully treated with alpha-blockers followed by surgical resection.
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Tafreshi et al. (2018) conducted a case report in Extra-adrenal pheochromocytoma presenting as inverse takotsubo-pattern cardiomyopathy (n=1). Surgical resection and alpha-blockers was evaluated on Symptom resolution and normalization of ejection fraction. Surgical resection of an extra-adrenal pheochromocytoma, preceded by alpha-blockade, resulted in complete resolution of symptoms and normalization of ejection fraction at 1-year follow-up.
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