Endomyocardial biopsy in a patient with hemorrhagic pheochromocytoma presenting as inverted Takotsubo cardiomyopathy
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A 29-year-old female patient presented with shock and dyspnea due to heart failure and pulmonary edema. Echocardiography indicated excessive contraction limited to the left ventricular apex and akinesis of the basal and middle ventricle, which were confirmed by emergency left ventriculography. The finding was diagnostic of inverted Takotsubo cardiomyopathy. An abdominal computed tomography scan showed a tumor in the left adrenal gland with a central low-density area, and the plasma and urinary catecholamines were strikingly elevated. Taken together, these findings suggested the presence of a hemorrhagic pheochromocytoma. A myocardial biopsy in the very acute stage on the day of admission revealed neutrophilic infiltration and contraction-band necrosis, which was indistinguishable from the previously reported pathology in the acute phase of idiopathic Takotsubo cardiomyopathy without pheochromocytoma. The diagnosis of pheochromocytoma in this case was confirmed 7 weeks later by surgical removal of the left adrenal gland with massive hemorrhage at the center of the pheochromocytoma. The marked similarity of the endomyocardial pathology between this case and cases with idiopathic Takotsubo cardiomyopathy strongly points to catecholamine excess as a common causality for Takotsubo cardiomyopathy with or without pheochromocytoma.
KeywordsPheochromocytoma Takotsubo cardiomyopathy Endomyocardial biopsy Shock
The authors thank Dr Hiroyuki Uchiyama for providing invaluable suggestions during the acute-phase management, Dr Yoshihiko Ikeda for constructive discussion regarding pathology, and Dr Osamu Ishizuka for providing the 131I-metaiodobenzylguanidine scintigraphy image and the photograph of the resected tumor. We are also grateful to Professor Hau C. Kwaan, Northwestern Medical Faculty Foundation, Chicago, and Robert Pastorek for editorial assistance.
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