98%
921
2 minutes
20
Wild-type transthyretin amyloidosis (ATTRwt) is an age-associated systemic disorder characterized by extracellular deposition of misfolded transthyretin amyloid fibrils, leading to progressive organ dysfunction. Cardiac involvement is common and may result in restrictive cardiomyopathy, arrhythmias, and conduction disturbances, including left bundle branch block (LBBB). Although pharmacological therapy with transthyretin stabilizers, such as tafamidis, has been shown to reduce mortality in patients with ATTRwt cardiac amyloidosis (ATTRwt-CA), the role of device-based therapies, such as cardiac resynchronization therapy (CRT), remains controversial, particularly in patients with coexisting conduction abnormalities. We report the case of an 81-year-old woman diagnosed with ATTRwt-CA who presented with symptomatic heart failure and LBBB. Tafamidis therapy was initiated to improve her exercise tolerance and to reduce her risk of mortality. Owing to the presence of LBBB and evidence of mechanical dyssynchrony, CRT was also introduced in an effort to prevent the deterioration of her heart failure with reduced ejection fraction. Device optimization was guided by gated myocardial perfusion imaging, performed using single photon emission computed tomography (SPECT), which enabled a detailed assessment of her left ventricular synchrony and informed CRT programming. Following CRT implantation and optimization, the patient exhibited marked symptomatic improvement, including resolution of her exertional dyspnea and fatigue. Objective assessments demonstrated improved left ventricular contractility and reverse remodeling, suggesting a favorable response to CRT. This case underscores the potential value of CRT in selected patients with ATTRwt-CA and conduction system disease, particularly when mechanical dyssynchrony is evident. Furthermore, it highlights the utility of nuclear imaging modalities such as SPECT in guiding CRT optimization in this unique patient population. Prospective studies are warranted to better define the indications, predictors of response, and long-term outcomes of CRT in patients with ATTRwt-CA.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC12223460 | PMC |
http://dx.doi.org/10.7759/cureus.87203 | DOI Listing |
Heart Rhythm O2
August 2025
Cardiac Electrophysiology Section, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania.
Background: Cardiac amyloidosis (CA) is characterized by atrial myopathy, which predisposes patients to atrial fibrillation (AF) and other atrial arrhythmias (AA). Although catheter ablation of AA is effective in the general population, its efficacy and safety in patients with CA remain unclear.
Objective: The study aimed to evaluate outcomes in patients with CA undergoing catheter ablation for typical atrial flutter (TAFL) and left atrial (LA) arrhythmias and to assess the presence and influence of LA low-voltage areas (LVA) in the latter.
Proteins
September 2025
School of Advanced Sciences and Languages (SASL), VIT Bhopal University, Sehore, Madhya Pradesh, India.
The mechanisms driving amyloid assembly have long intrigued structural biologists, as they offer insights into systemic fibrotic changes and the dynamic behavior of transthyretin (TTR) aggregation, crucial for developing amyloid-targeted therapies. In TTR-associated amyloidosis, amyloid fibrils form via destabilization of the tetramer into dimers and monomers. While many TTR mutations have been studied, the atomistic impact of multiple mutations on amyloid transthyretin (ATTR) self-assembly remains underexplored.
View Article and Find Full Text PDFJ Am Soc Echocardiogr
September 2025
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.
Background And Aims: Light chain cardiac amyloidosis (AL-CA), wild-type-transthyretin cardiac amyloidosis (ATTRwt-CA) and hereditary type-transthyretin cardiac amyloidosis (ATTRv-CA) have distinct presentations, clinical courses, and prognosis. To identify differentiating echocardiographic features and their prognostic significance, we investigated a large cohort of patients with CA.
Methods: In this multi-site cohort study, CA diagnosis was verified according to guidelines.
Amyloid
September 2025
Institute of Systems, Integrative and Molecular Biology, University of Liverpool, Liverpool, UK.
Background: Cross-seeding and co-assembly of multiple amyloid species are increasingly recognised in various organs and amyloidoses. Medin and wild-type transthyretin (TTR) both form age-related amyloid deposits and have been identified within the aortic wall. Given the emerging role of amyloid in aortic disease, this study investigates the potential colocalisation of TTR and medin in the aorta.
View Article and Find Full Text PDFJ Peripher Nerv Syst
September 2025
Mayo Clinic Department of Neurology, Rochester, Minnesota, USA.
Aim: To report a novel case of biopsy-proven, mass spectrometry-confirmed, wild-type transthyretin amyloidosis (ATTRwt) in nerve.
Methods: The patient was identified and evaluated in the peripheral nerve clinic. Our nerve laboratory's pathology database and the literature were searched for prior evidence of pathologically confirmed cases of ATTRwt.