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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2025; 35(03): 463-465
DOI: 10.1055/s-0044-1800882
Case Report

Isolated Right Atrial Enhancement with Atrial Standstill: An Uncommon Presentation of Emery–Dreifuss Muscular Dystrophy

Basavaraj Biradar
1   Department of Imaging Sciences and Interventional Radiology, Sree Chitra Institute of Medical Sciences, Trivandrum, Kerala, India
,
Jineesh Valakkada 
2   Department of Gastrointestinal Radiology, Sree Chitra Institute of Medical Sciences, Trivandrum, Kerala, India
,
Anoop Ayyappan 
1   Department of Imaging Sciences and Interventional Radiology, Sree Chitra Institute of Medical Sciences, Trivandrum, Kerala, India
,
Ajay Alex 
1   Department of Imaging Sciences and Interventional Radiology, Sree Chitra Institute of Medical Sciences, Trivandrum, Kerala, India
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Funding None.
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Abstract

Emery–Dreifuss muscular dystrophy (EDMD) is a rare inherited syndrome that affects muscles, joints, and the heart. The classic clinical triad includes early joint contractures, slowly progressive muscle weakness, and cardiac abnormalities. The common cardiac manifestations include conduction disturbances, systolic dysfunction, and dilated cardiomyopathy and may be associated with left ventricular noncompaction with an increased risk of thromboembolic events. Conduction disturbances include atrial arrhythmias, atrial standstill, complete heart block, or ventricular tachyarrhythmia. Cardiac magnetic resonance imaging (CMR) can help in the diagnosis of this condition by identifying chamber dilatation, systolic dysfunction, and late gadolinium enhancement of the atrium. Additional MRI finding of paraspinal muscle atrophy, an important finding in this muscular dystrophy, helped in reaching a confident imaging diagnosis. Our case in this article highlights the clinical and CMR findings of this rare condition.

Keywords

cardiac MR - atrial dilatation - stroke - muscular atrophy


Publikationsverlauf

Artikel online veröffentlicht:
17. Dezember 2024

© 2024. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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