Endocrine Abstracts

Background: Pheochromocytomas are catecholamine-producing neuroendocrine tumors, arising from the adrenal medulla or from paraganglionic chromafin tissue. Their clinical presentation, mainly attributable to the increased catecholamine production and it is highly variable. We herein present a case of a 42-year-old female patient with recurrent episodes of Takotsubo cardiomyopathy due to a pheochromocytoma.

Case presentation: The patient presented two years earlier to local hospital’s emergency department with chest pain and vomiting. The ECG showed sinus tachycardia, mild ST-segment elevation in leads I and aVL, and ST depression in leads II, III, aVF and V4-V6. An echocardiogram revealed marked hypokinesia of the midsegment and apical segment of the left (LV) ventricle with severely depressed LV function (LVEF): ≤30%. The patient underwent coronary angiography, which excluded the presence of significant coronary artery disease. Cardiac magnetic resonance (CMR) imaging, revealed myocardial edema with no late gadolinium enhancement. The patient was discharged on ramipril and carvedilol with a diagnosis of Takotsubo cardiomyopathy. A follow-up CMR study revealed normal LV systolic function with complete resolution of the myocardial edema and no late gadolinium enhancement. Medical treatment was stopped 6 months later and the patient remained asymptomatic. Two years later, the patient was admitted with acute heart failure following an episode of increased hypertension and was transferred to our hospital for further treatment Her echocardiogram revealed severe LV dysfunction (LVEF: 20%) with global, severe hypokinesia. After receiving medical supportive treatment, the patient exhibited a marked recovery of the LV function. During further evaluation, the patient underwent abdominal MRI, which revealed a solid, nodular lesion of 63 mm × 55 mm × 56 mm at the right adrenal gland. Metanephrine, normetanephrine, and vanillylmandelic acid levels in 24-h urine were elevated (9696 mcg/24 h, 683 mcg/24 h, and 40 mg/24 h, respectively) and consistent with pheochromocytoma. The patient was transferred to the Endocrinology Unit and was started on phenoxybenzamine, and 30 days later she underwent a laparoscopic right adrenalectomy. Pathological examination confirmed the diagnosis of pheochromocytoma. Patient re-evaluation during follow-up with scintigraphy and metanephrine measurement did not reveal any abnormal findings.

Conclusion: Patients with a clinical picture of acute myocardial infarction with Nonobstructive Coronary Arteries should always be investigated for pheochromocytoma/ paraganglioma.