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Circulation. 2000;102:e11-e13

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(Circulation. 2000;102:e11.)
© 2000 American Heart Association, Inc.


Circulation Electronic Pages

Pheochromocytoma-Induced Cardiomyopathy

Vamsi K. Mootha, MD; Jeremy Feldman, MD; Finn Mannting, MD; Gayle L. Winters, MD; Wendy Johnson, MD

From the Departments of Medicine, Cardiology, Nuclear Medicine, and Pathology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Mass.

Correspondence to Vamsi K. Mootha, MD, Department of Medicine, Brigham and Women’s Hospital, 75 Francis St, Boston, MA 02115.


*    Introduction
up arrowTop
*Introduction
 
A34-year-old woman presented with recurrent 15-minute episodes of palpitations, lightheadedness, and chest tightness. ECGs obtained during these episodes revealed striking global T-wave inversions that resolved spontaneously 2 hours after each episode (Figure 1Down). Echocardiography revealed severe left ventricular dysfunction with an estimated ejection fraction of 20%. Cardiac catheterization demonstrated angiographically normal coronary arteries. A 24-hour urine collection for catecholamines showed an epinephrine level of 227 µg (normal, 2 to 24 µg) and a metanephrine level of 3803 µg (normal, 95 to 475 µg). Abdominal/pelvic MRI identified a right adrenal mass (Figure 2Down). 123I-MIBG scintigram showed marked uptake in a single location corresponding to the right adrenal gland (Figure 3Down). The patient was diagnosed with an epinephrine-secreting pheochromocytoma and underwent an uncomplicated right adrenalectomy (Figure 4Down). Three months after surgery, the patient was asymptomatic. A repeat echocardiogram revealed normal ventricular function, and urine catecholamines remained negative.



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Figure 1. A, Baseline ECG obtained while patient was asymptomatic. B, ECG captured during a symptomatic episode reveals diffuse T-wave inversions.



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Figure 2. T2-weighted MRI reveals 3.2x2.8x3.6-cm high-signal lesion in right adrenal gland.



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Figure 3. Twelve-millimeter coronal slices of 123I-MIBG abdominal scintigram. Note marked uptake below liver at level of right adrenal gland.



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Figure 4. Right adrenalectomy surgical specimen containing pheochromocytoma. A, Gross appearance of bisected 32-g adrenalectomy specimen containing well-circumscribed 3.8-cm tumor with adjacent compressed uninvolved adrenal gland (top right). Necrosis is present, consisting of a 2.5x1.8-cm yellow area within mass surrounded by hemorrhagic rim. B, Portion of tumor before (left) and after (right) immersion in potassium dichromate solution demonstrating characteristic chromaffin reaction (brown) of pheochromocytoma. C, Microscopic appearance of pheochromocytoma consisting of relatively uniform cells with abundant cytoplasm and centrally placed nuclei separated into clusters by vascularized stroma.


*    Footnotes
 
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.





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Google Scholar
Right arrow Articles by Mootha, V. K.
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PubMed
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Right arrow Articles by Mootha, V. K.
Right arrow Articles by Johnson, W.
Related Collections
Right arrow Electrophysiology
Right arrow Biochemistry and metabolism
Right arrow Other heart failure
Right arrow Myocardial cardiomyopathy disease