ArticleJACC. Case reports2026
Recurrent Nonischemic Left Ventricular Aneurysms: A Cardiac MRI-Driven Diagnosis in a DES Variant Carrier.
Article in JACC. Case reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundCardiac magnetic resonance (CMR) is the gold-standard technique for characterizing nonischemic structural cardiomyopathies, offering tissue characterization and precise localization of myocardial injury. CASE SUMMARY: A 24-year-old man presented with recurrent chest discomfort and tachyarrhythmias. Initial CMR revealed an apical left ventricular aneurysm with imaging features consistent with a true aneurysm and normal coronary arteries, successfully treated surgically. Five years later, recurrent ventricular tachycardia prompted re-evaluation; CMR identified a new inferolateral basal aneurysm in a region previously normal on postoperative imaging. Coronary computed tomography angiography again showed normal epicardial coronary arteries. Surgical patch repair and implantable cardioverter-defibrillator placement were performed. Genetic testing revealed a pathogenic desmin (DES) c.785A>T (p.Glu262Val) variant and additional variant of unknown significance in VCL and CDH2. DISCUSSION: The identification of a pathogenic DES variant suggests a potential underlying susceptibility to structural remodeling, although causality cannot be confirmed. TAKE-HOME MESSAGES: CMR was crucial in identifying sequential, anatomically distinct left ventricular aneurysms. The combination of recurrent nonischemic aneurysms and a pathogenic DES variant suggests a possible cytoskeletal genetic cardiomyopathy with arrhythmic risk.
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