Background: Amyloidosis is a multi-organ disease of emerging significance in the field of cardiology. Chylothorax, a specific form of pleural effusion characterized by lymphatic fluid accumulation in the pleural cavity, is an extremely rare manifestation of amyloidosis. Notably, only five cases of chylothorax related to cardiac amyloidosis have been reported worldwide, all in amyloid light chain (AL) amyloidosis. No cases have been documented in amyloid transthyretin (ATTR) amyloidosis. Furthermore, elevated levels of serum carbohydrate antigen (CA) 125 have been associated with a poor prognosis in patients with AL cardiac amyloidosis.
Case Summary: We report the case of an 85-year-old Austrian man with pronounced left ventricular hypertrophy, monoclonal gammopathy, and a history of atrial fibrillation. Further examinations, including myocardial biopsy, confirmed the diagnosis of ATTR cardiac amyloidosis. A significant right-sided pleural effusion was also observed. Thoracocentesis diagnosed chylothorax, confirmed by lymphangiography. Elevated CA 125 levels were found in both serum and pleural fluid, with no other findings suspicious for malignancy. The patient underwent a short break in oral anticoagulation and received prophylactic low-molecular-weight heparin for myocardial biopsy, thoracocentesis, and lymphangiography. However, they died a few days later due to an embolic stroke.
Discussion: At this time, we can only speculate about the pathomechanism of chylothorax development in the context of amyloidosis. We recommend further investigation of similar cases to deepen understanding of the underlying causes and identify potential treatment strategies. Additionally, the utility of CA 125 as a prognostic marker in ATTR amyloidosis needs further investigation.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11694664 | PMC |
| http://dx.doi.org/10.1093/ehjcr/ytae634 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
FOUR QUESTIONS ABOUT ATRIAL FIBRILLATION IN CARDIAC AMYLOIDOSIS. WHY IS THIS ARRHYTHMIA DIFFERENT FROM ALL OTHER ARRHYTHMIAS?
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 PDFConduction disease in Cardiac Amyloidosis Patients: A Case series suggesting a role for Left Bundle Branch Area Pacing.
Indian 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 PDFSerum Biomarkers in Transthyretin Amyloidosis: An Overview of Neurofilaments, Cardiac, Renal, and Gastrointestinal Involvement.
Neurol 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 PDFBasic Science and Pathogenesis.
Alzheimers 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.
Basic Science and Pathogenesis.
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.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!