Korean Circ J.  2010 Apr;40(4):204-207. 10.4070/kcj.2010.40.4.204.

Endocardial Fibroelastosis in a 57-Year-Old Transplant Recipient

• Lee M
• Park SJ
• Kim MY
• Bae SY
• Chung HJ
• Kwon GY
• Choe YH
• Jeon ES

• KMID: 2145559
• DOI: http://doi.org/10.4070/kcj.2010.40.4.204

Abstract

Endocardial fibroelastosis (EFE) is characterized by deposition of collagen and elastin leading to ventricular hypertrophy and diffuse endocardial thickening. Here we report (for the first time in Korea) the case of a EFE presenting with heart failure. The patient was a 57-year-old woman who had complained of dyspnea on exertion {New York Heart Association (NYHA) functional class 3} and abdominal distension at the time of hospital admission. Echocardiography showed severe diastolic dysfunction with normal systolic function. On MRI, the contrast-enhanced delayed myocardial image demonstrated hyperenhancement in the endocardium. Owing to progressive heart failure, the patient was transplanted. Histological examination of the explanted heart showed irregularly thickened endocardium with fibrosis and elastosis in the both ventricles, compatible with the diagnosis of EFE.

Keyword

Endocardial fibroelastosis; Cardiomyopathy, restrictive; Cardiac transplantation

MeSH Terms

Cardiomyopathy, Restrictive
Collagen
Dyspnea
Echocardiography
Elastin
Endocardial Fibroelastosis
Endocardium
Female
Fibrosis
Heart
Heart Failure
Heart Transplantation
Humans
Hypertrophy
Middle Aged
Transplants
Collagen
Elastin

Figure

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  Fig. 1 On the chest X-ray, bilateral pleural effusion was observed.

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  Fig. 2 On cardiac MRI, the contrast-enhanced delayed myocardial image demonstrated endocardial hyperenhancement (arrows) in diffuse areas of both ventricles. A: short-axis view. B: four-chamber view.

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  Fig. 3 Gross and microscopic examination of the explanted heart. A: explanted heart specimen, cut along a basal ventricular short-axis plane, showing significant whitish endocardial thickening in both ventricles. B: a low-power photomicrograph showing areas of interstitial fibroelastosis (arrows) (Hematoxylin and Eosin staining ×100).

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