TY - JOUR
T1 - Cardiac Diagnoses and Long-Term Outcomes in Ring-Like Late Gadolinium Enhancement Evaluated by Cardiac Magnetic Resonance
AU - Filomena, Domenico
AU - Vandenberk, Bert
AU - Dresselaers, Tom
AU - Willems, Rik
AU - Masci, Pier Giorgio
AU - Robyns, Tomas
AU - Bogaert, Jan
N1 - © The Author(s) 2025. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact [email protected] for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact [email protected].
PY - 2025/2/18
Y1 - 2025/2/18
N2 - AIMS: Non-ischemic ring-like late gadolinium enhancement (LGE) in the left ventricle (LV) detected by cardiac magnetic resonance (CMR) is an emerging biomarker associated with adverse outcomes. Data regarding ring-like LGE are limited to small patient cohorts. We aimed to assess the prevalence of ring-like LGE, its association with morpho-functional phenotypes, aetiologic background, and prognostic implications.METHODS AND RESULTS: This single-center observational retrospective study included consecutive patients undergoing LGE-CMR between 2002 and 2024. Ring-like LGE was defined as continuous enhancement in ≥3 adjacent segments. Ischemic and amyloid cardiomyopathies were excluded. Clinical records were reviewed for etiologic diagnosis and clinical outcomes. The primary endpoint was a composite of all-cause mortality, heart transplantation, or LV assist device implantation. The secondary endpoint included sustained ventricular tachycardia, appropriate implantable cardioverter-defibrillator therapies, or sudden cardiac death.Among 14,091 unique patients who underwent LGE-CMR, ring-like LGE was identified in 152 patients (1.1%) with a median number of 10 segments, mostly involving the inferolateral segments. The most frequent morpho-functional phenotypes were dilated and non-dilated LV cardiomyopathy. Genetic testing identified likely pathogenic/pathogenic variants in 59 (58.4%) patients, affecting both desmosomal and non-desmosomal genes. Inflammatory cardiomyopathy was diagnosed in 15.8%. Other rare aetiologies included genetic neuromuscular diseases and inborn errors of metabolism. Primary and secondary endpoints occurred in 17.8% and 17.1%, respectively, over a median follow-up of 3 years.CONCLUSION: Ring-like LGE is an uncommon, non-disease-specific feature found in various morpho-functional CMP phenotypes. It is associated with frequent genetically determined aetiologies and a high burden of adverse outcomes.
AB - AIMS: Non-ischemic ring-like late gadolinium enhancement (LGE) in the left ventricle (LV) detected by cardiac magnetic resonance (CMR) is an emerging biomarker associated with adverse outcomes. Data regarding ring-like LGE are limited to small patient cohorts. We aimed to assess the prevalence of ring-like LGE, its association with morpho-functional phenotypes, aetiologic background, and prognostic implications.METHODS AND RESULTS: This single-center observational retrospective study included consecutive patients undergoing LGE-CMR between 2002 and 2024. Ring-like LGE was defined as continuous enhancement in ≥3 adjacent segments. Ischemic and amyloid cardiomyopathies were excluded. Clinical records were reviewed for etiologic diagnosis and clinical outcomes. The primary endpoint was a composite of all-cause mortality, heart transplantation, or LV assist device implantation. The secondary endpoint included sustained ventricular tachycardia, appropriate implantable cardioverter-defibrillator therapies, or sudden cardiac death.Among 14,091 unique patients who underwent LGE-CMR, ring-like LGE was identified in 152 patients (1.1%) with a median number of 10 segments, mostly involving the inferolateral segments. The most frequent morpho-functional phenotypes were dilated and non-dilated LV cardiomyopathy. Genetic testing identified likely pathogenic/pathogenic variants in 59 (58.4%) patients, affecting both desmosomal and non-desmosomal genes. Inflammatory cardiomyopathy was diagnosed in 15.8%. Other rare aetiologies included genetic neuromuscular diseases and inborn errors of metabolism. Primary and secondary endpoints occurred in 17.8% and 17.1%, respectively, over a median follow-up of 3 years.CONCLUSION: Ring-like LGE is an uncommon, non-disease-specific feature found in various morpho-functional CMP phenotypes. It is associated with frequent genetically determined aetiologies and a high burden of adverse outcomes.
U2 - 10.1093/ehjci/jeaf055
DO - 10.1093/ehjci/jeaf055
M3 - Article
C2 - 39964814
SN - 2047-2404
JO - European heart journal. Cardiovascular Imaging
JF - European heart journal. Cardiovascular Imaging
ER -