Circulation. 1996;94:2302-2303
(Circulation. 1996;94:2302.)
© 1996 American Heart Association, Inc.
Cardiac Hemochromatosis
Edward L. Passen, MD;
E. Rene Rodriguez, MD;
Alexander Neumann, BS;
Carmela D. Tan, MD;
Joseph E. Parrillo, MD
the Department of Medicine, Section of Cardiology (E.L.P., E.R.R., A.N., J.E.P.), and the Department of Pathology (E.R.R., C.D.T.), Rush-Presbyterian–St Luke's Medical Center, Chicago, Ill.
Correspondence to Edward L. Passen, MD, Associates in Cardiology, Ltd, Good Samaritan Hospital, Professional Building, 3825 S Highland Ave, Tower 1, Suite 4H, Downers Grove, IL 60515.
Cardiac hemochromatosis was found in a 46-year-old Caucasian man who had paroxysmal atrial fibrillation/flutter and congestive heart failure accompanied by liver failure and skin hyperpigmentation. Cardiac evaluation with echocardiography and cardiac catheterization, including right ventricular endomyocardial biopsy, revealed severe systolic and diastolic myocardial dysfunction with abundant iron deposits in the myocytes. These pathological findings of marked iron deposition in the myocardium can result in clinical congestive heart failure from both systolic and diastolic myocardial dysfunction.
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Figure 1. A, Simultaneous ECG tracing (top) of atrial fibrillation with right atrial pressure tracing (bottom) showing an elevated mean pressure with a rapid "y descent" consistent with restrictive physiology. B, Simultaneous ECG tracing (top) with a "dip and plateau" in the right ventricular pressure tracing (bottom; same scale and magnification as in A), also suggestive of restrictive physiology, although the rapid ventricular rate somewhat masks the late diastolic plateau. C, Transmitral pulsed Doppler echocardiography demonstrating a rapid deceleration slope with a calculated deceleration time of 80 ms, consistent with significant restrictive physiology. D and E, Apical four-chamber views in diastole (D) and in systole (E), demonstrating moderate biventricular enlargement with markedly reduced systolic performance (ejection fraction, 18%). In addition, note the marked biatrial enlargement.
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Figure 2. A, Light micrograph of the endomyocardial biopsy showing marked accumulation of yellow-brown pigment in the perinuclear regions of the myocytes (hematoxylin-eosin, x800). B, Polarization microscopy of the same section as shown in A. There is birefringence of the pigment within the myocytes, appearing as highlighted areas around the perinuclear regions (polarized hematoxylin-eosin, x800). C, Light micrograph showing marked iron deposition, as evidenced by the perinuclear and cytoplasmic blue granules (Perls' stain, x800). D, Electron micrograph showing irregular, electron-dense, granular deposits within lysosomes (siderosomes or siderin granules) surrounding the myocyte nucleus (uranyl acetate–lead citrate, x10 000). E, Electron micrograph showing the membrane-bound nature of the siderin granules containing electron-dense particles measuring 6 nm in average diameter and embedded in an electron-lucent matrix (uranyl acetate–lead citrate, x25 000).
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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, MC 4-265, Houston, TX 77030.
