Circulation. 2000;102:e11-e13
(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 Womens Hospital, Harvard Medical School, Boston,
Mass.
Correspondence to Vamsi K. Mootha, MD, Department of Medicine, Brigham and Womens Hospital, 75 Francis St, Boston, MA 02115.
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Introduction
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Top
Introduction
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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 1

). 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 2

).
123I-MIBG scintigram
showed marked uptake in a
single location corresponding to the
right adrenal gland (Figure 3

). The patient was diagnosed with
an
epinephrine-secreting pheochromocytoma and underwent an
uncomplicated
right adrenalectomy (Figure 4

). 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 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.
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Footnotes
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The editor of Images in Cardiovascular Medicine is Hugh A. McAllister,
Jr, MD, Chief, Department of Pathology, St Lukes 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 Lukes Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.