Abstract
WEBSITE FEATURE
A 59-year-old Caucasian woman presented for the evaluation of chest pain. Her history was notable for asthma, intermittent eosinophilia with normal bone marrow biopsy, and intermittent angioedema. Coronary catheterization for the chest-pain study revealed nonobstructive coronary artery disease and a blunt left ventricular (LV) apex on ventriculography (Fig. 1). Cardiac magnetic resonance (CMR) revealed a normally contractile but hypertrophied LV, with blunt apex in 2008 and again in 2010 (Fig. 2). Delayed-enhancement images showed diffuse subendocardial hyperenhancement of the left and right ventricles, with a large thrombus in the apex of the left (Fig. 3). There was no evidence of valvular abnormality or other congenital malformation. Endomyocardial biopsy revealed markedly thickened fibroelastic endocardium with abundant elastic fibers (Figs. 4 and 5), which supported the diagnosis of endocardial fibroelastosis. In a 3-year follow-up CMR scan, the characteristics of the earlier findings remained unchanged. The patient had been placed on an anticoagulative regimen upon the initial discovery of mural thrombus and remained asymptomatic from a cardiac perspective at the time of her follow-up scan.
Fig. 1 Left ventriculogram shows a blunt apex (arrow).
Real-time motion image is available at www.texasheart.org/journal.
Fig. 2 Cardiac magnetic resonance images (steady-state free-precession cine in 4-chamber views). A) View in 2008 shows a small left ventricle with a blunt apex. Increased signal intensity in the pericardial space can represent fat or fluid; in this case, the signal properties of the delayed-enhancement image (using a phase-sensitive inversion-recovery sequence) confirmed the presence of fat. See Figure 3A for the delayed-enhancement image. B) View in 2010 shows a small left ventricle with a blunt apex.
Motion image of Figure 2A is available at www.texasheart.org/journal.
Fig. 3 Cardiac magnetic resonance images (delayed-enhancement, 4-chamber views). A) In 2008, diffuse hyperenhancement was seen in the subendocardium of both ventricles, with a thrombus (arrow) in the left ventricular apex. The presence of pericardial fat is confirmed by a homogeneous bright signal in the pericardial space, as opposed to a lack of signal in the case of pericardial effusion. B) In 2010, diffuse hyperenhancement was seen in the subendocardium of both ventricles, with a thrombus (arrow) in the left ventricular apex.
Fig. 4 Photomicrograph of endomyocardial biopsy sample shows markedly thickened fibroelastic endocardium (H & E, orig. ×40).
Fig. 5 Photomicrograph. Van Gieson preferentially stains the abundant elastic fibers. Orcein (dark blue) stains the elastic fibers of the endocardium (orig. ×40).
Comment
Endocardial fibroelastosis (a term that was coined in the early 1940s1) is primarily a disease of infancy and early childhood,2 with rare occurrences in young adulthood.3–5 On the basis of LV size, primary endocardial fibroelastosis can present in the more common dilated form or the rarer contracted form (with restrictive physiology). Endocardial fibroelastosis is characterized by endocardial thickening due to zealous fibroelastic proliferation. Other notable features include mural thrombi and valvular abnormalities due to thickened and shortened papillary muscles and chordae tendineae. Common clinical presentations include LV dysfunction, congestive heart failure, and sudden cardiac death.6 Endocardial fibroelastosis can also occur secondary to congenital heart diseases, especially hypoplastic left-heart syndrome or LV obstructive lesions. Infection, malignancy, nutritional deficiencies, and eosinophilia have been suggested as possible causes. We believe ours to be a rare case of endocardial fibroelastosis in an adult, causally related to intermittent eosinophilia. It should be noted, however, that a temperate variant of eosinophilic heart disease, such as Loeffler's endomyocarditis,7,8 cannot be completely ruled out, because eosinophilic infiltration is commonly absent on endomyocardial biopsy, due to sampling limitations and stage of the disease.
Supplementary Material
Footnotes
Address for reprints: Jie J. Cao, MD, MPH, 101 Northern Blvd., Greenvale, NY 11548
E-mail: Jane.Cao@chsli.org
Dr. Gupta is now at the Division of Cardiovascular Diseases, University of Iowa Hospitals and Clinics, Iowa City, Iowa.
References
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