Multifocal Myocardial Necrosis: A Distinctive Cardiac Lesion in Cystic Fibrosis, Lipomatous Pancreatic Atrophy, and Keshan Disease

Abstract

Multifocal myocardial necrosis (MMN) is an unusual cardiomyopathy of childhood, characterized by multiple patchy areas of myocardial fiber necrosis/fibrosis involving mainly the middle part of the left ventricle, but also, to a lesser extent, the right ventricle and the atria. These necrotic lesions are isolated and are not accompanied by an inflammatory reaction or vascular alterations. They are responsible for acute cardiac failure. MMN lesions are observed in various pathologic conditions including cystic fibrosis of the pancreas, pancreatic lipomatous hypoplasia/atrophy, malnutrition due to extensive intestinal resection with subsequent total parenteral feeding, and in Keshan disease. MMN is the main and the most characteristic feature of Keshan disease, an endemic and idiopathic condition affecting Chinese rural children. The causes and mechanisms of MMN presently are unknown. However, the presence of similar cardiac lesions in such different pathological conditions suggests the role of a selective deficiency of a hypothetical extrinsic factor (selenium, molybdenum iodide, other), probably crucial for the metabolism of the myocardial fiber.

Transcriptome analysis of the response to chronic constant hypoxia in zebrafish hearts

“…Selenoprotein P (SEPP1) is a heparin-binding protein that appears to be associated with endothelial cells and has been implicated as an oxidant defense in the extracellular space. Human populations that are selenium deficient are susceptible to the development of Keshan disease, a potentially fatal form of cardiomyopathy (Nezelof et al 2002 ). SEPP1 expression was found to be increased in our study indicating a potential protective mechanism against oxidative stress in the heart.…”

Exercise-induced hypoxemia and cardiac arrhythmia in cystic fibrosis

“…The authors speculated that either an insufficient blood supply to the myocardium and/or regional myocardiopathy were responsible for the perfusion anomalies. Further reports indicate fibrotic changes in the myocardium of patients with CF [17,18]. Anyhow, neither De Wolf et al [16] nor any of the other studies we could find report on arrhythmias during exercise in CF.…”

Myocardial function in patients with Shwachman–Diamond syndrome: Aspects to consider before stem cell transplantation

“…Lethal neonatal cardiac manifestations have been associated with SDS in several reports 10–16 (Table I). Histopathology has mostly shown myocardial necrosis or fibrosis.…”