Delayed-Enhanced Cardiac MRI for Differentiation of Fabry's Disease from Symmetric Hypertrophic Cardiomyopathy

被引：85

作者：

De Cobelli, Francesco ^{[1
,2
]}

Esposito, Antonio ^{[1
,2
]}

Belloni, Elena ^{[1
]}

Pieroni, Maurizio ^{[3
]}

Perseghin, Gianluca ^{[4
,5
]}

Chimenti, Cristina ^{[6
]}

Frustaci, Andrea ^{[6
]}

Del Maschio, Alessandro ^{[1
,2
]}

机构：

[1] Ist Sci San Raffaele, Dept Radiol, I-20132 Milan, Italy

[2] Univ Vita Salute San Raffaele, I-20132 Milan, Italy

[3] Univ Cattolica Sacro Cuore, Dept Cardiol, I-00168 Rome, Italy

[4] Ist Sci San Raffaele, Sect Nutr & Metab, I-20132 Milan, Italy

[5] Univ Milan, Ctr Phys Exercise Hlth & Wellness, CDI Fac Exercise Sci, Milan, Italy

[6] Univ Roma La Sapienza, Dept Heart & Great Vessels Attilio Reale, Rome, Italy

来源：

AMERICAN JOURNAL OF ROENTGENOLOGY | 2009年 / 192卷 / 03期

关键词：

cardiac MRI; delayed-enhanced MRI; Fabry's disease; hypertrophic cardiomyopathy; CARDIOVASCULAR MAGNETIC-RESONANCE; PREVALENCE; VARIANT;

D O I：

10.2214/AJR.08.1201

中图分类号：

R8 [特种医学]; R445 [影像诊断学];

学科分类号：

1002 ; 100207 ; 1009 ;

摘要：

OBJECTIVE. Fabry's disease may be difficult to differentiate from symmetric hypertrophic cardiomyopathy. Our aim was to compare the myocardial location and distribution patterns of delayed enhancement between patients with Fabry's disease who are affected by symmetric myocardial hypertrophy and patients with symmetric hypertrophic cardiomyopathy in order to identify a specific sign to best differentiate the two diseases. CONCLUSION. Patients with Fabry's disease-related hypertrophy showed left ventricular (LV) delayed enhancement with a typical and consistently found pattern characterized by the involvement of the inferolateral basal or mid basal segments and a mesocardial distribution that spared the subendocardium. This pattern seems to be specific to Fabry's disease; in fact, patients with symmetric hypertrophic cardiomyopathy had variable locations and distributions of delayed enhancement. These observations may contribute to identifying Fabry's disease as a specific cause of symmetric hypertrophy.

引用

页码：W97 / W102

页数：6

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