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. 2024 Dec 2;7(12):e2450251.
doi: 10.1001/jamanetworkopen.2024.50251.

Nonischemic Cardiac Manifestations in VEXAS Syndrome

Marie Robert  1 , Alexis Mathian  2 , Valentin Lacombe  3 , Hervé Lobbes  4 , Léopold Adélaïde  5 , Maelle Le Besnerais  6 , Jean-Nicolas Dacher  7 , Benjamin Terrier  8 , Arsène Mékinian  9 , Rim Bourguiba  10 , Sophie Georgin-Lavialle  11 ; FRENVEX Group
Collaborators, Affiliations

Nonischemic Cardiac Manifestations in VEXAS Syndrome

Marie Robert et al. JAMA Netw Open. .
No abstract available

Plain language summary

This case series reports on the clinical presentation, laboratory findings, imaging characteristics, treatments, and outcomes of nonischemic cardiac manifestations in patients with VEXAS syndrome with confirmed pathogenic UBA1 alterations.

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Conflict of interest statement

Conflict of Interest Disclosures: None reported.

Figures

Figure.
Figure.. Cardiac Magnetic Resonance Imaging of Patient 3 Showing Myocardial Infarctions and Necrosis Sequalae
A, Cine sequence, with midventricular short-axis view at end systole showing severe thinning of the inferolateral wall of the left ventricle (white arrows). B, Delayed enhancement sequence performed 10 minutes after gadolinium injection (4-chamber view), with subendocardial enhancement of the left ventricular wall (white arrowheads), nodular enhancement of the apical septum (white arrow), and enhancement of the right ventricular apex (black arrowhead). C, Delayed enhancement sequence performed 10 minutes after gadolinium injection (2-chamber view), with nodular foci of enhancement of the anterior wall of the left ventricle (arrowheads). D, T1 mapping performed 12 minutes after gadolinium injection, with midventricular short-axis view showing decreased signal of the inferolateral wall of the left ventricle (arrow) due to gadolinium retention and nodular focus of decreased signal in the anterior septum (arrowhead).
See this image and copyright information in PMC

References

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