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Year : 2021  |  Volume : 31  |  Issue : 3  |  Page : 131-136

Regional strain pattern and correlation with cardiac magnetic resonance imaging in fabry disease

1 Department of Medicine, Division of Cardiology, University of California, Irvine, CA, USA
2 Department of Pediatrics, Division of Genetic and Genomic Medicine, University of California, Irvine, CA, USA

Correspondence Address:
Virginia E Kimonis
Department of Pediatrics, Division of Genetics and Genomic Medicine, University of California Irvine, 101 The City Drive South, ZC4482, Orange, CA 92868
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcecho.jcecho_119_20

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Background: Cardiovascular disease is the most common cause of death among Fabry disease patients, who carry significantly increased risk for heart failure and sudden cardiac death. Echocardiographic strain imaging and cardiac MRI are important clinical tools for early detection of cardiomyopathy before onset of systolic or diastolic dysfunction. However, studies on these imaging modalities are limited among Fabry patients. Aim and Objective: To evaluate echocardiographic strain pattern and correlation with cardiac MRI in Fabry disease. Materials and Methods: We performed a detailed analysis of global longitudinal strain and correlation with cardiac MRI finding in 9 patients diagnosed with Fabry disease. Results: Despite normal left ventricular ejection fraction, basal and mid inferior segments are more likely to demonstrate strain abnormalities compared to other regions. Additionally, increased interventricular septal and left ventricular posterior wall thickness are correlated with greater strain abnormalities. Finally, MRI evidence of fibrosis and infiltration are detected among most patients with strain abnormalities, but in some cases, strain imaging were able to detect early evidence of cardiomyopathy even before MRI was fully able to detect the change. Basal and mid inferoseptal segment strain abnormalities are early signs of developing cardiomyopathy among patients with Fabry disease. Conclusion: Though cardiac MRIs are critical tools for detection of myocardial infiltration and scarring, these findings may not always be detectable in early phases of the disease. Multiple imaging modalities maybe considered in monitoring and evaluation of cardiomyopathy in Fabry disease.

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