The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in a patient with Anderson-Fabry disease

被引：144

作者：

Moon, James C.

Sheppard, Mary

Reed, Emma

Lee, Phillip

Elliott, Perry M.

Pennell, Dudley J.

机构：

[1] Royal Brompton Hosp, CMR Unit, London SW3 6NP, England

[2] Royal Brompton Hosp, Dept Histopathol, London SW3 6NP, England

[3] UCL Natl Hosp Neurol & Neurosurg, London WC1N 3BG, England

[4] Heart Hosp, London, England

来源：

JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE | 2006年 / 8卷 / 03期

关键词：

cardiomyopathy; hypertrophy; magnetic resonance imaging; Anderson-Fabry disease;

D O I：

10.1080/10976640600605002

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Anderson-Fabry Disease (AFD) is a storage disease that mimics hypertrophic cardiomyopathy. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance occurs in approximately 50% of patients in the basal inferolateral LV wall, but how an intracellular storage disease causes focal LGE is unknown. We present a whole-heart histological validation that LGE is caused by focal myocardial collagen scarring. This scarring may be the substrate for electrical re-entry and sudden arrhythmic death. The reasons for this distribution of fibrosis are unclear, but may reflect inhomogeneous left ventricular wall stress.

引用

页码：479 / 482

页数：4

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