Cardiac hemochromatosis simulating constrictive pericarditis

Wasserman, Alan G.; Richardson, D. W.; Baird, Charles L.; Wyso, E. M.

doi:10.1016/0002-9343(62)90299-1

Cited by 56 publications

(12 citation statements)

References 18 publications

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“…Neither serum iron, transferrin saturation, serum ferritin, the amount of iron in the liver nor the presence of endocrine organ involvement correlated with the presence of stainable iron in the heart. These observations are consistent with the clinical observation of a nonlinear progression in the symptoms of cardiac hemosiderosis (4,7,8,9), and patients with documented iron overload are well known to experience rapid cardiac deterioration. This may be related either to the accumulation of a critical amount of iron in a chronically ironloaded heart or to factors which facilitate cardiac iron deposition.…”

Section: Discussionsupporting

confidence: 89%

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Myocardial iron grading by endomyocardial biopsy. A clinico‐pathologic study on iron overloaded patients

Barosi

Arbustini²,

Gavazzi

et al. 1989

European J of Haematology

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Endomyocardial biopsy was performed in 13 patients with primary or secondary iron overload. Prussian blue staining showed visible iron in the biopsy fragments of 8 out of 13 patients. Because of the inhomogeneity of iron deposition in the biopsy fragments, a semi‐quantitative myocardial iron grading system was used in which the percentage of Perls' positive cells on 4 to 6 biopsy fragments was averaged from each case. The presence of stainable iron in the myofibrils was not predictable from serum iron, transferrin saturation, serum ferritin or liver iron grading, nor from evidence of endocrine dysfunction. In patients with Perls' positive material in the myocardium, there was a significant correlation between the endomyocardial iron grade and serum iron and transferrin saturation. These results suggest that other factors besides the body iron load determine cardiac iron deposition. The fact that myocardial siderosis was documented only in patients with hepatic cirrhosis, irrespective of the hepatic iron load, suggests that severe liver damage may be a prerequisite for the accumulation of iron in the heart.

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Section: Discussionsupporting

confidence: 89%

“…Autopsy studies have demonstrated that the extent of body iron load, as evaluated by hepatic iron deposition, does not correlate well with cardiac iron accumulation (6). Moreover, the rapidly progressive nature of cardiac failure often ob- (1,7,8,9), points to a non-linear progression of cardiac hemosiderosis.…”

mentioning

confidence: 99%

Myocardial iron grading by endomyocardial biopsy. A clinico‐pathologic study on iron overloaded patients

Barosi

Arbustini²,

Gavazzi

et al. 1989

European J of Haematology

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“…Pericardial constriction or tamponade may lead to rapid clinical deterioration. 49 Pericarditis and angina without coronary artery disease have been described. 50 the entire cardiac conduction system may be involved with the sinoatrial node being the least common site of iron deposition.…”

Section: Symptomatic Hemochromatosismentioning

confidence: 99%

“…54 the most common disturbances in the rhythm are atrial tachyarrhythmias, mainly paroxysmal atrial fibrillation, followed in frequency by ventricular premature beats and tachycardia. 49 First degree atrioventricular (Av) blocks and supraventricular arrhythmias correlate with the extent of iron deposition in the atrial myocardium. 48 the frequency of ventricular arrhythmias increases with lv dilation and systolic dysfunction.…”

Section: Symptomatic Hemochromatosismentioning

confidence: 99%

Cardiac Involvement in Hemochromatosis

Gulati

Harikrishnan

Palaniswamy

et al. 2014

Cardiology in Review

116

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Cardiac hemochromatosis or primary iron-overload cardiomyopathy is an important and potentially preventable cause of heart failure. This is initially characterized by diastolic dysfunction and arrhythmias and in later stages by dilated cardiomyopathy. Diagnosis of iron overload is established by elevated transferrin saturation (>55%) and elevated serum ferritin (>300 ng/mL). Genetic testing for mutations in the HFE (high iron) gene and other proteins, such as hemojuvelin, transferrin receptor, and ferroportin, should be performed if secondary causes of iron overload are ruled out. Patients should undergo comprehensive 2D and Doppler echocardiography to evaluate their systolic and diastolic function. Newer modalities like strain imaging and speckle-tracking echocardiography hold promise for earlier detection of cardiac involvement. Cardiac magnetic resonance imaging with measurement of T2* relaxation times can help quantify myocardial iron overload. In addition to its value in diagnosis of cardiac iron overload, response to iron reduction therapy can be assessed by serial imaging. Therapeutic phlebotomy and iron chelation are the cornerstones of therapy. The average survival is less than a year in untreated patients with severe cardiac impairment. However, if treated early and aggressively, the survival rate approaches that of the regular heart failure population.

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“…Patients' initial presentation is often exertional shortness of breath as a result of left ventricular diastolic dysfunction secondary to a restrictive pathophysiology. This condition may later progress to a dilated cardiomyopathy with left ventricular systolic dysfunction (23,27,28). Iron accumulation occurs in the ventricular myocardium before the atrial myocardium (22).…”

Section: Clinical Presentationmentioning

confidence: 99%

Iron Overload Cardiomyopathy

Gujja

Rosing

Tripodi

et al. 2010

Journal of the American College of Cardiology

249

100

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The prevalence of Iron Overload Cardiomyopathy (IOC) is increasing. The spectrum of symptoms of IOC is varied. Early in the disease process, patients may be asymptomatic while severely overloaded patients can have terminal heart failure complaints that are refractory to treatment. It has been shown that early recognition and intervention may alter outcomes. Biochemical markers and tissue biopsy, that have traditionally been used to diagnose and guide therapy, are not sensitive enough to detect early cardiac iron deposition. Newer diagnostic modalities such as MRI are noninvasive and can assess quantitative cardiac iron load. Phlebotomy and chelating drugs are suboptimal means of treating IOC; hence the roles of gene therapy, hepcidin, and CCBs are being actively investigated. There is a need for the development of clinical guidelines in order to improve the management of this emerging complex disease.

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Cardiac hemochromatosis simulating constrictive pericarditis

Cited by 56 publications

References 18 publications

Myocardial iron grading by endomyocardial biopsy. A clinico‐pathologic study on iron overloaded patients

Myocardial iron grading by endomyocardial biopsy. A clinico‐pathologic study on iron overloaded patients

Cardiac Involvement in Hemochromatosis

Iron Overload Cardiomyopathy

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