Midmural Fibrosis of Left Ventricle Due to Selenium Deficiency
A 38-year-old man with Crohn’s disease had been receiving total parenteral nutrition for 16 years, from the age of 22 years. At the age of 28 years, he had rapidly developing heart failure and ventricular premature beats associated with selenium (Se) deficiency. Laboratory data included a serum Se concentration of 62 μg/L (normal value, 80 to 230 μg/L), an erythrocyte Se concentration of 93 μg/L (normal value, 100 to 194 μg/L), and an erythrocyte glutathione peroxidase activity of 5.4 U/g hemoglobin (normal value, 25.3 to 45.3 U/g hemoglobin). Supplements of Se improved his condition but did not normalize the left ventricular dysfunction.
The patient was given Se supplements and was free from symptoms of heart failure for 11 years, but the echocardiographic findings gradually deteriorated. He was admitted with congestive heart failure at the age of 38 years. He improved with the administration of digitalis and diuretics and left the hospital. Five days after discharge, he suddenly died at his home. At autopsy, the heart was markedly enlarged and weighed 490 g. Microscopic examination of the myocardium disclosed scattered and irregular areas of myocardial loss and fibrous replacement that were localized to the midmural regions in the left ventricular free wall (Figures 1⇓ and 2⇓). In the interventricular septum, fibrosis was linear and restricted to the right ventricular side. The subendocardial region and the right ventricular free wall were relatively spared.
Johnson et al1 described an “occidental” patient in whom Se deficiency became associated with dilated cardiomyopathy; autopsy showed similar subepicardial fibrosis. We speculate that the midmural change is characteristic of cardiomyopathy related to Se deficiency. Once fully developed, the left ventricular dysfunction may be irreversible even after use of Se supplements.
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.
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