Pheochromocytoma is a rare catecholamine-secreting tumor with variable clinical manifestations. A possible cardiac presentation is acute left ventricular wall motion abnormality in the heart also known as the Takotsubo syndrome. Takotsubo stress cardiomyopathy was first described in 1990 in Japan and current knowledge remains limited. We report a case of pheochromocytoma-induced Takotsubo syndrome. A 43-year-old woman acutely developed nausea, severe chest pain, and dyspnoea during a strenuous hiking-trip in the mountains. She arrived at our hospital in cardiac shock with need for invasive ventilation and hemodynamic monitoring. The ECG revealed an ectopic atrial rhythm, poor R-wave progression, and a prolonged corrected QT-interval. Chest X-ray showed pulmonary oedema. Troponin level was 2.42 μg/l (normally <0.005 ng/l) and NT-proBNP was 39.061 ng/l (normally <125 ng/l). Echocardiography documented severely impaired left ventricular function with basal and mid segmental akinesis and preserved contractility of the apex. Coronary angiography ruled out significant coronary disease. Ventriculography confirmed inverted Takotsubo cardiomyopathy. Because of refractory high blood pressure, a diagnostic work-up was performed. 24-hour fractionated urinary metanephrine and normetanephrine levels were 7.483 and 8.895 μg/g creatinine respectively (normally <240 μg/g creatinine and <600 μg/g creatinine). Contrast-enhanced total body CT scan identified a left adrenal heterogenous mass of 59×56 mm. The diagnosis of pheochromocytoma was suggested. Adequate alpha-adrenergic blockade was ensured and laparoscopic adrenalectomy was performed. Pathological examination revealed nests of polygonal cells with eosinophilic cytoplasm. Immunohistochemical staining was strongly positive for chromogranin A and S100, confirming the diagnosis of pheochromocytoma. A rapid cardiac recovery was observed after tumor removal with complete recovery of ventricular wall motion and function. Pheochromocytoma-induced Takotsubo syndrome is very rare. Many stress factors have been identified as possible causes, and hypercatecholaminemia due to a secreting pheochromocytoma is one of them. This case presents a clear example of a physical trigger preceding the Takotsubo syndrome. Pheochromocytoma-induced Takotsubo syndrome may deteriorate more rapidly than other Takotsubo syndrome cases, with possible catastrophic presentations as in our case. We suggest screening for an underlying pheochromocytoma in Takotsubo syndrome, especially in younger patients, complicated or recurrent cases, in atypical (inverted/basal or global) Takotsubo subtypes and when other suggestive symptoms of pheochromocytoma are present.
21 Oct 2019
Belgian Endocrine Society