Cardiac Hemochromatosis
1996; Lippincott Williams & Wilkins; Volume: 94; Issue: 9 Linguagem: Inglês
10.1161/01.cir.94.9.2302
ISSN1524-4539
AutoresEdward Passen, E. René Rodríguez, Alexander Neumann, Carmela D. Tan, Joseph E. Parrillo,
Tópico(s)Iron Metabolism and Disorders
ResumoHomeCirculationVol. 94, No. 9Cardiac Hemochromatosis Free AccessResearch ArticleDownload EPUBAboutView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticleDownload EPUBCardiac Hemochromatosis Edward L. Passen, E. Rene Rodriguez, Alexander Neumann, Carmela D. Tan and Joseph E. Parrillo Edward L. PassenEdward L. Passen 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. , E. Rene RodriguezE. Rene Rodriguez 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. , Alexander NeumannAlexander Neumann 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. , Carmela D. TanCarmela D. Tan 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. and Joseph E. ParrilloJoseph E. Parrillo 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. Originally published1 Nov 1996https://doi.org/10.1161/01.CIR.94.9.2302Circulation. 1996;94:2302–2303Cardiac 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.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.Download figureDownload PowerPoint 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.Download figureDownload PowerPoint 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, ×800). 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, ×800). C, Light micrograph showing marked iron deposition, as evidenced by the perinuclear and cytoplasmic blue granules (Perls' stain, ×800). D, Electron micrograph showing irregular, electron-dense, granular deposits within lysosomes (siderosomes or siderin granules) surrounding the myocyte nucleus (uranyl acetate–lead citrate, ×10 000). 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, ×25 000).FootnotesCorrespondence 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. Previous Back to top Next FiguresReferencesRelatedDetailsCited By Wang B, Zhang Y, Sun N, Gu S, Ding F, Xu S, Zhou H and Liu Y (2018) MRI-measured myocardial iron load in patients with severe diabetic heart failure, Clinical Radiology, 10.1016/j.crad.2017.10.012, 73:3, (324.e1-324.e7), Online publication date: 1-Mar-2018. Rozwadowska K, Daniłowicz-Szymanowicz L, Fijałkowski M, Sikorska K, Gałąska R, Kozłowski D, Gruchała M and Raczak G (2018) Can two-dimensional speckle tracking echocardiography be useful for left ventricular assessment in the early stages of hereditary haemochromatosis?, Echocardiography, 10.1111/echo.14141, 35:11, (1772-1781), Online publication date: 1-Nov-2018. Dettmeyer R (2018) Vascular, Cardiac Valve, and Metabolic Diseases Forensic Histopathology, 10.1007/978-3-319-77997-3_14, (357-380), . Kanzaki Y, Yuki M, Yamamura K, Narumi Y and Ishizaka N (2016) Is cardiac and hepatic iron status assessed by MRI T2* associated with left ventricular function in patients with idiopathic cardiomyopathy?, Heart and Vessels, 10.1007/s00380-016-0814-1, 31:12, (1950-1959), Online publication date: 1-Dec-2016. Kilias A, Klingel K, Gawaz M, Kramer U and Seizer P (2015) A patient with a rare cause of elevated troponin I, Clinical Research in Cardiology, 10.1007/s00392-015-0864-x, 104:9, (794-797), Online publication date: 1-Sep-2015. Dettmeyer R (2011) Vascular, Cardiac Valve, and Metabolic Diseases Forensic Histopathology, 10.1007/978-3-642-20659-7_14, (283-302), . Demant A, Schmiedel A, Büttner R, Lewalter T and Reichel C (2007) Heart failure and malignant ventricular tachyarrhythmias due to hereditary hemochromatosis with iron overload cardiomyopathy, Clinical Research in Cardiology, 10.1007/s00392-007-0568-y, 96:12, (900-903), Online publication date: 1-Dec-2007. Daniłowicz-Szymanowicz L, Świątczak M, Sikorska K, Starzyński R, Raczak A and Lipiński P (2021) Pathogenesis, Diagnosis, and Clinical Implications of Hereditary Hemochromatosis—The Cardiological Point of View, Diagnostics, 10.3390/diagnostics11071279, 11:7, (1279) Schmid J and Venkatesh M (2013) Haemochromatosis (cardiac manifestations) Radiopaedia.org, 10.53347/rID-26134 November 1, 1996Vol 94, Issue 9 Advertisement Article InformationMetrics Copyright © 1996 by American Heart Associationhttps://doi.org/10.1161/01.CIR.94.9.2302 Originally publishedNovember 1, 1996 Advertisement
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