Background: Light chain amyloidosis (AL) is a rare plasma cell dyscrasia associated with poor survival especially in the setting of heart failure. Late gadolinium enhancement (LGE) on cardiac MRI was recently found to correlate with myocardial amyloid deposition but the prognostic role is not established. The aim is to determine the prognostic significance of LGE in AL by comparing long term survival of AL patients with and without LGE.
Methods: Twenty nine consecutive patients (14 females; 62 +/- 11 years) with biopsy-proven AL undergoing cardiac MRI with gadolinium as part of AL workup were included. Survival was prospectively followed 29 months (median) following MRI and compared between those with and without LGE by Kaplan-Meier and log-rank analyses.
Results: LGE was positive in 23 subjects (79%) and negative in 6 (21%). Left ventricular ejection fraction was 66 +/- 17% in LGE-positive and 69 +/- 12% in LGE-negative patients (p = 0.8). Overall 1-year mortality was 36%. On follow-up, 14/23 LGE-positive and none of LGE-negative patients died (log rank p = 0.0061). Presenting New York Heart Association heart failure class was also associated with poor survival (p = 0.0059). Survival between two LGE groups stratified by heart failure class still showed a significant difference by a stratified log-rank test (p = 0.04).
Conclusion: Late gadolinium enhancement is common and is associated with poor long-term survival in light chain amyloidosis, even after adjustment for heart failure class presentation. The prognostic significance of late gadolinium enhancement in this disease may be useful in patient risk-stratification.
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http://dx.doi.org/10.1186/1756-6649-9-5 | DOI Listing |
Int J Cardiovasc Imaging
January 2025
Michigan Medicine, University Hospital, Floor B1 Reception C 1500 E Medical Center Dr SPC 5030, Ann Arbor, MI, 48109, USA.
Anderson-Fabry disease (AFD) is a X-linked lysosomal storage disorder that can result in cardiac dysfunction including left ventricular hypertrophy (LVH) and conduction abnormalities (Frontiers in cardiovascular medicine vol. 10) [1]. The manifestations of AFD in women may be isolated to one organ and occur late in life due to the random inactivation of the X chromosome.
View Article and Find Full Text PDFHeart Rhythm
January 2025
Department of Cardiovascular Medicine, Section of Cardiovascular Imaging, Cleveland Clinic Foundation, Cleveland, OH. Electronic address:
Background: Better risk stratification is needed to evaluate patients with non-ischemic cardiomyopathy (NICM) for prophylactic implantable cardioverter-defibrillators (ICD). Growing evidence suggests cardiac magnetic resonance imaging (CMR) may be useful in this regard.
Objective: We aimed to determine if late-gadolinium enhancement (LGE) seen on CMR (dichotomized as none/minimal <2% vs significant ≥2%) predicts appropriate ICD therapies (primary endpoint) and/or all-cause mortality/transplant/left-ventricular assist device (LVAD) implantation (secondary endpoint) in NICM patients.
J Clin Med
December 2024
Department of Pediatric Cardiology, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, University of Health Sciences, Istanbul 34303, Turkey.
: Cardiac magnetic resonance (CMR) plays a central role in the diagnosis and follow-up of acute myocarditis (AM). In this study, we aimed to evaluate baseline and follow-up CMR findings and associated factors in children with AM. : A retrospective analysis of CMR in pediatric patients with clinical presentations suggestive of myocarditis was performed.
View Article and Find Full Text PDFInt J Mol Sci
December 2024
Second Department of Cardiology, Hippokration General Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece.
Atrial fibrosis is a hallmark of atrial cardiomyopathy and plays a pivotal role in the pathogenesis of atrial fibrillation (AF), contributing to its onset and progression. The mechanisms underlying atrial fibrosis are multifaceted, involving stretch-induced fibroblast activation, oxidative stress, inflammation, and coagulation pathways. Variations in fibrosis types-reactive and replacement fibrosis-are influenced by patient-specific factors such as age, sex, and comorbidities, complicating therapeutic approaches.
View Article and Find Full Text PDFJ Electrocardiol
December 2024
Crown Princess Victoria Children's Hospital, Dept of Biomedical and Clinical Sciences, Dept of Pediatrics, Linköping University, Sweden; Pediatric Heart Centre, Skåne University Hospital and Dept of Clinical Sciences, Lund University, Sweden. Electronic address:
Background: Myocardial fibrosis, expressed as late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR), is an important risk factor for malignant cardiac events in hypertrophic cardiomyopathy (HCM). However, CMR is not easily available, expensive, also needing intravenous access and contrast.
Objective: To determine if derived vectorcardiographic spatial QRS-T angles, an aspect of advanced ECG (A-ECG), can indicate LGE to appropriately prioritize young HCM-patients for CMR.
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