(Circulation. 1998;97:2093-2094.)
© 1998 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Amyloidosis With Cardiac Involvement
Ho-Joong Youn, MD;
Jang Seong Chae, MD;
Kyo Young Lee, MD;
; Soon Jo Hong, MD
From the Department of Internal Medicine (H.-J.Y., J.S.C., S.J.H.), and
Clinical Pathology (K.Y.L.), The Catholic University of Korea.
Correspondence to Ho-Joong Youn, MD, Department of Internal Medicine, The Catholic University of Korea, St Mary's Hospital, #62, Yoido-Dong, Youngdungpo-Gu, Seoul, 150-010, Korea.
A 59-year-old woman
was admitted because of lethargy. She had been treated for heart
failure for 5 years before admission, which progressed to include
lower-extremity edema as well as orthopnea and nocturnal dyspnea.
The serial ECGs and echocardiographic findings are
summarized in Figs 1
and 2. Forearm skin, rectal (Fig 3), and
endomyocardial biopsies (Fig 4) were performed.
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Figure 1. Serial ECG changes. Top, Sinus rhythm at 80 bpm
with first-degree AV block (PR interval, 0.22 second) and right
bundle-branch block. Middle, Sinus rhythm at 90 bpm with first-degree
AV block (PR interval, 0.24 second), right bundle-branch block, and
left axis deviation. Bottom, Complete AV block at 48 bpm and low
voltage.
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Figure 2. Top, Serial two-dimensional
echocardiographic findings. Thickening of all
myocardial walls and valves; "patch amorphous, high-intensity
echoes" in left ventricular myocardium; and a
small pericardial effusion were noted (top left). Bottom,
Serial M-mode echocardiography showed gradual
increase of interventricular septal, left
ventricular posterior wall, and right
ventricular wall thickness.
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Figure 3. Lamina propria and mucosa of rectum (left) and
dermis of skin (right) showing yellow-green birefringence after Congo
red stain under polarizing microscopic examination (x100).
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Figure 4. Top left, Amyloid material stained red within the
cardiac muscle cells with Congo red stain (x100). Top right, Typical
yellow-green birefringence after Congo red stain under polarizing
microscopic examination (x100). Bottom, Positive reaction of amyloid
material between atrophic cardiac muscle cells with monoclonal murine
antibody of amyloid associated protein (x200).
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On the basis of our findings (Figs 1 through 4
), the diagnosis of
amyloidosis with cardiac involvement was confirmed. Placement of a
permanent pacemaker (VVI type) for the treatment of complete
atrioventricular block resulted in marked improvement
in dyspnea and lethargy.
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 Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1–267, Houston, TX 77030.
