Objectives: Left atrial (LA) function is an important marker of hemodynamic status in cardiac amyloidosis (CA), and its characterization may provide relevant prognostic information. We sought to assess the prevalence and prognostic impact of LA dysfunction by cardiac magnetic resonance (CMR) in patients with CA.
Methods: We performed CMR in 80 consecutive patients with CA, including 38 with AL (47%) and 42 with ATTR (53%). LA function was assessed by acquiring short axis cine steady-state free precession (SSFP) covering the entire chamber. The atrial emptying fraction (AEF) was calculated as the ratio between the difference of LA maximal and minimal volume to LA maximal volume, expressed as percentage. Severe atrial dysfunction was defined as AEF ≤ 14%.
Results: Mean AEF was 18% (13-35%). Overall, AEF ≤ 14% was present in 19 patients (24%), including 21% of those in sinus rhythm (SR) with no history of atrial fibrillation (AF). After a median of 3 years (IQR 2-4), 36 patients (44%) died of cardiac causes. Patients with AEF ≤ 14% showed increased cardiac mortality, with an independent OR of 4.2 (95 IC 2.1-8.2, P < 0.0001). Of note, AEF ≤ 14% was the stronger independent predictor of cardiac death. Patients in SR with AEF ≤ 14% had worse outcome than those with AF.
Conclusions: Severe impairment of LA contractile function was present in three-quarters of patients with CA, and was prevalent irrespective of CA etiology, both in the presence and absence of AF. Severe LA dysfunction was associated with an independent 4-fold increase in risk for cardiac death at three years.
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http://dx.doi.org/10.1016/j.ijcha.2020.100633 | DOI Listing |
Can J Cardiol
January 2025
Brigham and Women's Hospital Amyloidosis Program and Section of Cardiology, Brigham and Women's Hospital, Boston MA 02115 USA; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
AF is a common arrhythmia in cardiomyopathy, particularly when congestive heart failure is present. The neurohormonal activation in congestive heart failure may trigger fibrotic and other changes in the left atrium and the atrial stretch associated with heart failure may induce further atrial pathology and/ or directly trigger AF (8). By the time that patients with AF develop extensive fibrosis, the arrhythmia has been shown to be associated with a greater difficulty in maintaining sinus rhythm despite attempted ablation procedures.
View Article and Find Full Text PDFIndian Pacing Electrophysiol J
January 2025
Royal Jubilee Hospital, Vancouver Island Health Authority, British Columbia, Canada.
Transthyretin Cardiac amyloidosis (ATTR-CA) is an increasingly recognised cause of heart failure in our elderly patients with preserved ejection fraction. Patients with ATTR-CA who require permanent pacemaker implantation often have preserved ejection fraction and do not meet the clinical indication for cardiac resynchronization therapy (CRT). In these patients, left bundle branch area pacing (LBBAP) can be a reasonable option to maximise physiological activation of the left ventricle.
View Article and Find Full Text PDFNeurol Ther
January 2025
Department of Neuroscience, Università Cattolica del Sacro Cuore, Rome, Italy.
Hereditary transthyretin amyloidosis (ATTRv, v for variant) is a genetic disorder characterized by the deposition of misfolded transthyretin (TTR) protein in tissues, resulting in progressive dysfunction of multiple organs, including the nervous system, heart, kidneys, and gastrointestinal (GI) tract. Noninvasive serum biomarkers have become key tools for diagnosing and monitoring ATTRv. This review examines the role of available biomarkers for neurological, cardiac, renal, gastrointestinal, and multisystemic involvement in ATTRv.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
Vanderbilt Genetics Institute, Vanderbilt University Medical Center, Nashville, TN, USA.
Background: Genome-wide association studies (GWAS) in Alzheimer's disease (AD) leveraging endophenotypes beyond case/control diagnosis, such as brain amyloid β pathology, have shown promise in identifying novel variants and understanding their potential functional impact. In this study, we leverage two brain amyloid β pathology measurement modalities, PET imaging and neuropathology, to address sample size limitations and to discover novel genetic drivers of disease.
Method: We conducted a meta-analysis on an amyloid PET imaging GWAS (N = 7,036, 35% amyloid positive, 53.
Alzheimers Dement
December 2024
Alzheimer's Center at Lewis Katz School of Medicine, Temple University, Philadelphia, PA, USA.
Background: FDA-approved carbonic anhydrase inhibitors (CAIs) have been shown to attenuate Aβ pathology, neurodegeneration, and cerebrovascular dysfunction in models of Alzheimer's disease (AD) and cerebral amyloid angiopathy (CAA), suggesting a key role for CAs as a novel and previously unexplored target for AD therapy. Amyloid β accumulation severely impairs the cerebral neuro-signaling pathway with a progressive loss in neurotrophic factors (NTFs, i.e.
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