Ring-like late gadolinium enhancement pattern in antimalarial-induced cardiomyopathy

A 67-year-old woman with systemic lupus erythematosus (SLE) and 22 years of hydroxychloroquine therapy presented with NYHA III–IV dyspnoea, markedly elevated NT-proBNP and troponin T, and fragmented QRS with Q waves and ST-segment elevation. Cardiac magnetic resonance (CMR) showed left ventricular hypertrophy, reduced left ventricular ejection fraction (32%) and extensive midwall intramyocardial late gadolinium enhancement (LGE) forming a circumferential ring-like pattern (Figure 1, panels A–C). A 60-year-old woman with SLE and 31 years of hydroxychloroquine exposure presented with NYHA IV dyspnoea, high NT-proBNP and troponin T levels, and fragmented QRS. CMR demonstrated left ventricular hypertrophy, mildly reduced systolic function (50%) and extensive midwall ring-like LGE (Figure 1, panels E–G). A 65-year-old woman with SLE treated with hydroxychloroquine for 43 years presented with chest pain, cardiac biomarkers elevation, and ventricular ectopy. CMR revealed concentric hypertrophy, preserved ejection fraction (60%), mild pericardial effusion, and ring-like LGE (Figure 1, panels I–K). All three patients had unobstructed coronary arteries and underwent endomyocardial biopsy which results were consistent with antimalarial-induced cardiomyopathy, including diffuse myocyte vacuolization, cytoplasmic inclusions, interstitial fibrosis, and curvilinear bodies on electron microscopy (Figure 1, panels D, H, and L). Further details are provided in Supplementary material online. (A–C) Short-axis LGE images from patient #1 demonstrating extensive midwall enhancement forming a complete ring-like configuration with marked lateral and septal walls involvement. The dotted arrow corresponds to a nearly transmural basal inferolateral enhancement. (D) Corresponding endomyocardial biopsy (HES staining) showing vacuolization and myocytes ballooning (arrows), intracytoplasmic inclusions, and fibrosis (asterisk). (E–G) Short-axis LGE views from patient #2 reveal a diffuse midwall enhancement pattern, involving basal, mid-ventricular, and apical anteroseptal, inferoseptal, and lateral segments, reconstituting a circumferential ring-like aspect. The dotted arrow shows a nearly transmural basal inferolateral enhancement. (H) Corresponding endomyocardial biopsy (HES staining) showing vacuolization and myocytes ballooning with intracyoplasmic inclusions (arrows). (I–K) Short-axis LGE images from patient #3 show marked inferior and inferolateral nearly transmural enhancement combined with midwall septal anterior and inferior involvement, together producing a ring-like pattern. The dotted arrow shows a nearly transmural basal inferolateral enhancement. A mild pericardial effusion can also be seen. (L) Corresponding endomyocardial biopsy (electron microscopy) showing lamellar myeloid bodies (arrows) and a mitochondrion with disorganized cristae (asterisk). Across all three cases, CMR consistently disclosed a distinctive midwall ring-like LGE phenotype. This pattern, classically associated with inherited arrhythmogenic cardiomyopathies, has not previously been described in antimalarial-induced cardiomyopathy.1,2 Our observations suggest that hydroxychloroquine cardiotoxicity may converge towards a ring-like myocardial scar phenotype, representing a novel imaging signature of advanced disease. Supplementary material is available at European Heart Journal – Case Reports online. Levi-Dan Azoulay (Writing—review & editing [lead]), Alexis Mathian (Writing—review & editing [supporting]), Nadjia Kachenoura (Conceptualization, Validation [supporting]), Zahir Amoura ( Writing—review & editing [supporting]), and Alban Redheuil (Writing—review & editing [supporting]) We thank Dr. Rouvier for his valuable contributions to this study. Despite the best efforts of the authors, written consent for publication could not be obtained for these cases. Every effort has been made to anonymize the cases. The situation has been discussed with the editor. L-D.A. is a recipient of the Poste d’Accueil Inserm (Publicly funded PhD scholarship from the French National Institute of Health and Medical Research). This work was not funded by the industry. The de-identified data are available in Supplementary material Online.